Bispecific HER2 ligands for cancer therapy

10093740 ยท 2018-10-09

Assignee

Inventors

Cpc classification

International classification

Abstract

The invention relates to a bispecific HER2-targeting agent comprising a.) a first polypeptide ligand that binds to HER2 extracellular domain 1, b.) a second polypeptide ligand that binds to HER2 extracellular domain 4 and c.) a linker covalently attaching said first polypeptide ligand to said second polypeptide ligand.

Claims

1. A bispecific HER2 targeting agent comprising an amino acid sequence as set forth in SEQ ID 03, SEQ ID 04, SEQ ID 05, SEQ ID 06, SEQ ID 07, SEQ ID 08, SEQ ID 09, SEQ ID 58, or SEQ ID 59.

2. A method of treating a HER2-expressing cancer, comprising administering a bispecific HER2 targeting agent of claim 1 to a patient with a HER2-expressing cancer, thereby treating the HER2 cancer.

3. The bispecific HER2 targeting agent of claim 1, which comprises the amino acid sequence as set forth in SEQ ID 03.

4. A method of treating a HER2-expressing cancer, comprising administering a bispecific HER2 targeting agent of claim 3 to a patient with a HER2-expressing cancer, thereby treating the HER2 cancer.

5. The bispecific HER2 targeting agent of claim 1, which comprises the amino acid sequence as set forth in SEQ ID 04.

6. A method of treating a HER2-expressing cancer, comprising administering a bispecific HER2 targeting agent of claim 5 to a patient with a HER2-expressing cancer, thereby treating the HER2 cancer.

7. The bispecific HER2 targeting agent of claim 1, which comprises the amino acid sequence as set forth in SEQ ID 05.

8. A method of treating a HER2-expressing cancer, comprising administering a bispecific HER2 targeting agent of claim 7 to a patient with a HER2-expressing cancer, thereby treating the HER2 cancer.

9. The bispecific HER2 targeting agent of claim 1, which comprises the amino acid sequence as set forth in SEQ ID 06.

10. A method of treating a HER2-expressing cancer, comprising administering a bispecific HER2 targeting agent of claim 9 to a patient with a HER2-expressing cancer, thereby treating the HER2 cancer.

11. The bispecific HER2 targeting agent of claim 1, which comprises the amino acid sequence as set forth in SEQ ID 07.

12. A method of treating a HER2-expressing cancer, comprising administering a bispecific HER2 targeting agent of claim 11 to a patient with a HER2-expressing cancer, thereby treating the HER2 cancer.

13. The bispecific HER2 targeting agent of claim 1, which comprises the amino acid sequence as set forth in SEQ ID 08.

14. A method of treating a HER2-expressing cancer, comprising administering a bispecific HER2 targeting agent of claim 13 to a patient with a HER2-expressing cancer, thereby treating the HER2 cancer.

15. The bispecific HER2 targeting agent of claim 1, which comprises the amino acid sequence as set forth in SEQ ID 09.

16. A method of treating a HER2-expressing cancer, comprising administering a bispecific HER2 targeting agent of claim 15 to a patient with a HER2-expressing cancer, thereby treating the HER2 cancer.

17. The bispecific HER2 targeting agent of claim 1, which comprises the amino acid sequence as set forth in SEQ ID 58.

18. A method of treating a HER2-expressing cancer, comprising administering a bispecific HER2 targeting agent of claim 17 to a patient with a HER2-expressing cancer, thereby treating the HER2 cancer.

19. The bispecific HER2 targeting agent of claim 1, which comprises the amino acid sequence as set forth in SEQ ID 59.

20. A method of treating a HER2-expressing cancer, comprising administering a bispecific HER2 targeting agent of claim 19 to a patient with a HER2-expressing cancer, thereby treating the HER2 cancer.

21. A bispecific HER2 targeting agent comprising an amino acid sequence as set forth in SEQ ID 102, SEQ ID 103, SEQ ID 104, SEQ ID 105, SEQ ID 106, SEQ ID 107, SEQ ID 108, SEQ ID 109, or SEQ ID 110.

22. A method of treating a HER2-expressing cancer, comprising administering a bispecific HER2 targeting agent of claim 21 to a patient with a HER2-expressing cancer, thereby treating the HER2 cancer.

Description

SHORT DESCRIPTION OF THE FIGURES

(1) FIG. 1 shows the increased anti-tumor activity of bispecific targeting agents in cell proliferation assays. The Y axis shows cell viability in different indicated cell lines expressing HER2 after treatment with any of the agents identified in the X axis; A: AU565 (upper panel), BT474 (lower panel); B: HCC2218 (upper panel), HCC1419 (lower panel), C: SkBr3 (upper panel), ZR7530 (lower panel).

(2) FIG. 2 shows the quantification of cellular DNA content by flow cytometry in absence and presence of different anti-tumor agents.

(3) FIG. 3 shows the induction of apoptosis by bispecific targeting agents quantified by terminal transferase dUTP nick end labeling (TUNEL) assays and flow cytometry wherein the Y-axis shows fraction of positive cells after treatment with any of the agent identified in the X axis; A: AU565 cells (upper panel), BT474 cells (lower panel); B: HCC2218 cells (upper panel), HCC1419 cells (lower panel).

(4) FIG. 4 shows the Western blot analysis of HER2/HER3 signaling pathway, PI3K/AKT and MAPK pathway and downstream targets of cell cycle and apoptosis.

(5) FIG. 5 shows quantitative western blot analyses of the treatment time course measuring HER2/HER3 receptor expression and phosphorylation after treatment with anti-HER2 binding agents, wherein the Y axis shows normalized signal intensity of the western blot band, and the X axis the time after treatment of the respective anti-HER2 binding agent indicated in the respective legends; A: phosphoHER2 band in BT474 cells; B phospho-HER3 in BT474 cells.

(6) FIG. 6 shows the inhibition of ligand-stimulated growth by bispecific targeting agents in cell proliferation assays.

(7) FIG. 7 shows the pictorial summary of anti-HER2 targeting formats.

(8) FIG. 8 shows the anti-tumor activity of bispecific binding reagents quantified by cell proliferation assays, shown is the effect of different concentrations of anti-tumor agents on the cell viability.

(9) FIG. 9 shows the anti-tumor activity of all constructs that share a similar epitope on domain I of ECD HER2 in a cell proliferation assay, the Y-axis showing the viability of BT474 cells after treatment with any of the agents identified in the legend.

(10) FIG. 10 shows the anti-tumor activity of single binding agents. The Y-axis shows the viability of BT474 cells after treatment with any of the agents identified in the legend.

(11) FIG. 11 shows the effect of combination treatment of the single anti-HER2 binding agents on the cell viability (Y-axis) of BT474 cells after treatment with any of the agents identified in the legend.

(12) FIG. 12 shows the effect of different anti-tumor agents on the viability of trastuzumab-resistant cell lines in cell proliferation assays, the Y-axis showing the viability of the cell lines determined by absorbance of reduced XTT after treatment with any of the agents identified in the figure. Tested cell lines were A: MDA-MB-361; B: MDA-MB-453; C: Sk-Ov-3; D: JIMT-1; and E: several XTT resistant cells lines as indicated.

(13) FIG. 13 shows the effect of trastuzumab and pertuzumab on the anti-tumor activity of the bispecific targeting agents in cell proliferation assays. Data presentation as in FIG. 11, wherein the Y axis shows the viability of cells treated with the anti-tumor agents as indicated in the X axis. A: XTT competing with the activity of the indicated bivalent ligands; B: combination of the indicated agents with trastuzumab or pertuzumab.

(14) FIG. 14 shows the anti-tumor activity of different anti-tumor agents in cell proliferation assays. Data presentation as in FIG. 11.

(15) FIG. 15 shows the results of an ELISA with bispecific targeting agents and pertuzumab. The Y-axis shows the concentration of the agents identified in the legend bound to HER2 in presence of pertuzumab.

(16) FIG. 16 shows the competitive binding of G3 and H14 with trastuzumab. The Y-axis shows the percent binding of the agents identified in the legend to domain 4 of HER2 in presence of trastuzumab.

(17) FIG. 17 shows the binding affinity, binding stoichiometry and binding mode of single binding units and bispecific binding agent to HER2 on the surface of cancer cells; A and B, on-rate determination of single binding agents; C, on-rate determination of bispecific binding agents; D, off-rate determination of single and bispecific binding agents; MFI mean fluorescence intensity.

(18) FIG. 18 shows the dissociation from the surface of BT474 cells (A) and the anti-tumor activity (B-G) of single binding agents and bispecific binding agents. A: median fluorescence intensities of fluorescently labeled agents bound to the BT474 surface are plotted as function of dissociation time; B-G: The Y-axes show the viability of BT474 (B-D, F,G) or MCF7 (E) cells after treatment with any of the agents identified in the legend.

(19) FIG. 19 shows the construction principle of the A21H_4D5LH_A21L anti-tumor agent (A) and a cartoon of the complete diabody construct (B) as expressed in CHO cells. Here heavy chain refers to the VH domain, light chain to the VL domain, GGGGS-linker to peptide linker with 4 glycine and 1 serine (eg. as set forth at positions 172-176 of SEQ ID NO. 3), 4GGGGS-linker to a linker peptide consisting of 4 repeats of 4 glycine and 1 serine (eg. as set forth at positions 168-187 of SEQ ID NO. 9).

(20) FIG. 20 shows the effect of different anti-tumor agents on the viability of BT474 cells in cell proliferation assays, the Y-axis showing the viability of the cell lines determined by absorbance of reduced XTT after treatment with any of the agents (100 nM) identified in the legend. Data were normalized to the control, which was set to 100%.

(21) FIG. 21 shows the effect of different anti-tumor agents on the viability of HCC1419 cells in cell proliferation assays, the Y-axis showing the viability of the cell lines determined by absorbance of reduced XTT after treatment with any of the agents (100 nM) identified in the legend. Data were normalized to the control, which was set to 100%.

(22) FIG. 22 shows the induction of apoptosis in BT474 cells by bispecific targeting agents quantified by terminal transferase dUTP nick end labeling (TUNEL) assays and flow cytometry.

(23) FIG. 23 shows the Western blot analysis of apoptosis as detected by the cleavage of Poly ADP Ribose Polymerase (PARP). GAPDH is a loading control.

(24) FIG. 24 shows the effect of different anti-tumor agents on the viability of BT474 cells in cell proliferation assays, the Y-axis showing the viability of the cell lines determined by absorbance of reduced XTT after treatment with any of the agents identified in the legend.

EXAMPLES

Example 1: Anti-Tumor Activity of the Bispecific Anti-HER2 Binding Agents in Comparison to Trastuzumab and Pertuzumab

(25) A XTT cell proliferation assay was performed with a panel of HER2 overexpressing cancer cell lines in 96-well tissue culture plates (FIG. 1). A defined number of cells were seeded in RPMI1640 medium containing 10% fetal calf serum (FCS). Cancer cells were treated for 4 days with 100 nM of anti-HER2 agents and controls. Measuring points were recorded in triplicates. XTT cell viability assays were developed according to the manufacturer's protocol. At a concentration of 100 nM, all anti-HER2 agents show maximal anti-tumor activity (titration not shown). The average of three data points is plotted with standard error. Data were normalized against the negative control on each plate, which corresponds to untreated cells (maximal growth). Bispecific targeting agents reduce cell growth of HER2-dependent cancer cells by 60-80%, while trastuzumab (hu4D5) reduces cell growth by only 20-60%. Bispecific targeting agents (926-FL-G3, 929-FL-H14) show consistently strong anti-tumor activity in all cell lines, while some cell lines show resistance against trastuzumab treatment. Sensitive cell lines can be roughly defined as HER2 dependent (e.g. HER2 overexpressing) and lacking any PI3K activating mutation.

(26) FIG. 2 shows that bispecific targeting agents block entrance into S-phase and induce accumulation in G.sub.0/1-Phase. BT474 cells were seeded 16 h before treatment in RPMI1640 containing 10% FCS. Anti-HER2 agents were added to a final concentration of 100 nM and cells were treated for 3 days. Afterwards, cells were fixed in 70% EtOH and stained with propidium iodide (PI). FACS measurements were gated to exclude cell debris in a forward vs. side scatter plot and 10.sup.4 events were recorded. PI fluorescence histograms were analyzed by FlowJo 7.2.5 software, and cell cycle distribution was fitted using the Dean-Jett-Fox algorithm, which excludes the apoptotic SubG1-population of cells. Treatment with bispecific targeting agents (926-FL-G3, 929-FL-H14) reduces S-phase and G.sub.2/M-phase content in HER2-dependent cancer cells. It was shown that trastuzumab (hu4D5) treatment induces cell cycle arrest by blocking entrance into S-phase of sensitive HER2 dependent cancer cell lines. Here it is shown that bispecific targeting agents also induce cell cycle arrest in trastuzumab sensitive cell lines.

(27) The terminal transferase dUTP nick end labeling (TUNEL) assay and quantification by flow cytometry was used to determine the portion of apoptotic cells upon treatment with anti-HER2 agents (FIG. 3). Cancer cells were seeded 16 h before treatment in RPMI1640 containing 10% FCS. Anti-HER2 agents (pertuzumab: hu2C4; trastuzumab: hu4D5; bispecific targeting agents: 926-FL-G3, 929-FL-H14; mock treatment: Off7-FL-Off7) were added to a final concentration of 100 nM and cells were treated for 3 days. Fractions of adherent and non-adherent cells were pooled. Cells were fixed in 2% paraformaldehyde, permeabilized in cold 0.1% sodium citrate containing 0.1% Triton X-100 for 2 min, washed three times with cold PBS and labeled with fluorescein-conjugated dUTP. FACS measurements were gated to exclude cell debris in a forward vs. side scatter plot and 10.sup.4 events were recorded. Measurements were plotted as an one parameter FL1 histogram plots (FITC fluorescence on the X-axis and counts on the Y-axis). Population of TUNEL positive (shift towards higher FL1) cells were quantified by one-dimensional regional gates which exclude TUNEL negative cells (auto fluorescence). Gates were applied according to negative control to exclude auto fluorescent cells. Treatment with bispecific targeting agents induces DNA degradation in HER2-dependent cancer cells, which is a hallmark of apoptosis. The number of TUNEL-positive cells correlates with the formation of a Sub-G1 population, as determined by cell cycle analysis (data not shown). The quantification shows 30- to 80-fold higher TUNEL signals for the bispecific binding agents than for trastuzumab or pertuzumab in HER2-dependent cancer cells.

(28) For Western blot analysis of the HER2/HER3 signalling pathway, PI3K/AKT and MAPK pathway and downstream targets of cell cycle and apoptosis, cancer cells were seeded 24 h before treatment in RPMI1640 containing 10% FCS. Anti-HER2 agents were added to a final concentration of 100 nM and cells were treated for 3 days. Afterwards, the fraction of detached apoptotic cells was collected and removed by centrifugation. Remaining attached cells were washed with cold PBS and scraped off into cold PBS_I (PBS containing protease inhibitors (Pefabloc, Leupeptin, Pepstatin, Marimastat) and phosphatase inhibitors (sodium orthovanadate, sodium metavanadate, sodium molybdate, -glycerol phosphate, sodium fluoride)) on ice. Both cell fractions were pooled and washed in PBS_I. Afterwards, cells were lysed in PBS_I containing 1% Triton X-100 for 30 min at 4 C. on a rocker, and cell lysates were centrifuged at 20,000 g for 20 min at 4 C. Protein concentrations of the respective cell lysates were determined by BCA assays and samples were taken up in lithium dodecyl sulfate (LDS) loading buffer containing -mercaptoethanol for complete reduction. Samples were heated for 5 min at 80 C. Samples were loaded on 10% SDS-PAGE and afterwards blotted on PVDF_FL membrane (Millipore) according to a BioRad protocol. After incubation with primary detection antibodies, western blots (FIG. 4.) were stained by secondary antibodies labeled with an infrared dye, and membranes were scanned on an Odyssey IR-fluorescence scanning system (LICOR). The following primary detection antibodies were used: Human Epidermal Growth Factor Receptor 2 (HER2); Phospho-Tyr 1248 Human Epidermal Growth Factor Receptor 2 (HER2-Y1248); Human Epidermal Growth Factor Receptor 3 (HER3); Phospho-Tyr 1289 Human Epidermal Growth Factor Receptor 3 (HER3-Y1289); Protein Kinase B (AKT); Phospho-Ser 473 Protein Kinase B (AKT-5473); p44/42 MAPK (ERK1/2); Phospho-Thr202/Tyr204 p44/42 MAPK (ERK1/2-T202/Y204); Cyclin-depended Kinase Inhibitor 1B (p27KIP1); CyclinD1 (CyclinD1); Poly ADP Ribose Polymerase (PARP); Bcl-2 Interacting Mediator of Cell Death (BIM); Glyceraldehyde 3-Phosphate Dehydrogenase (GAPDH).

(29) For quantitative western blot analysis of the time course treatments, BT474 cells were seeded 24 h before treatment in RPMI1640 containing 10% FCS. Anti-HER2 agents were added to a concentration of 100 nM and cells were treated for 3 days. Afterwards, the fraction of only loosely adherent cells was washed away with cold PBS. Attached cells were scraped off in cold PBS_I (PBS containing protease inhibitors (Pefabloc, Leupeptin, Pepstatin, Marimastat) and phosphatase inhibitors (sodium orthovanadate, sodium metavanadate, sodium molybdate, -glycerol phosphate, sodium fluoride)) on ice. Afterwards, cells were lysed in PBS_I containing 1% Triton X-100 for 30 min at 4 C. on a rocker and cell lysates were centrifuged at 20,000 g for 20 min at 4 C. Protein concentrations of the respective cell lysates were determined by BCA assays. HER2 receptor was immunoprecipitated by 901-FL-zHER2, a DARPin-affibody fusion construct, linked to Biosupport Ultra Link beads. HER2 receptor was depleted from BT474 cell lysate (corresponding to 1 mg protein in the lysate). Beads were washed three times with cold PBS_I. HER2 receptor was eluted from beads by heating to 80 C. for 5 min in LDS loading buffer containing -mercaptoethanol for complete reduction. HER3 samples were heated for 5 min at 80 C. in LDS loading buffer containing -mercaptoethanol for complete reduction. Samples were loaded on 10% SDS-PAGE and afterwards blotted on PVDF_FL membrane according to the BioRad protocol. Western blots were stained by secondary antibodies labeled with an infrared dye and membranes were scanned on an Odyssey IR-fluorescence scanning system (LICOR).

(30) Bispecific agents down-regulate phospho-HER2 levels consistently in all HER2-dependent cancer cells. Down-regulation of phospho-HER2 can correlate with down-regulation of HER2 expression level, which was observed in the fractions of apoptotic cells (FIG. 4). Constant HER2 expression levels were observed in the fraction of attached cells for e.g. BT474 and SkBr3, while phospho-HER2 levels were strongly reduced (FIG. 5). Therefore, down-regulation of HER2 expression can be observed in the apoptotic fraction of HER2-dependent cancer cells but is probably not the cause for induction of apoptosis. Rather, down-regulation of phosho-HER2 simultaneously with reduction of phosho-HER3 is the cause for induction of apoptosis. Down-regulation of phospho-HER3 can be observed after treatment with bispecific targeting agents and trastuzumab. Bispecific targeting agents show stronger down-regulation of phospho-HER3 than trastuzumab. Up-regulation of HER3 expression can be observed after treatment with bispecific targeting agents. A feedback loop sensing inhibition of phospho-AKT and, consequently, up-regulation of HER3 expression has been proposed. Bispecific targeting agents reduce phospho-AKT (downstream HER3) and phospho-ERK (downstream HER2) signaling simultaneously. Trastuzumab treatment mainly down-regulates phospho-AKT, while in ZR7530 cells, trastuzumab treatment leads to a down regulation of phospho-ERK. Cell cycle regulators p27KIP1 (inhibitor of cyclin-dependent kinases) is up-regulated and CyclinD1, which mediates G1/S-phase transition, is down-regulated in several HER2-dependent cancer cell lines. Again, inhibition of the cell cycle is not necessarily observed by bispecific targeting agents, but cell cycle arrest is observed in cell lines which are sensitive to trastuzumab treatment. BIM.sub.S up-regulation and PARP cleavage (up-regulation of PARP p89) is observed in all HER2-dependent cancer cell lines, which show induction of apoptosis after treatment with bispecific targeting agents. ZR7530 and BT474 cells show also PARP cleavage after treatment with trastuzumab, but bispecific targeting agents show consistently stronger signals.

(31) XTT cell proliferation assays were performed with BT474 cells in 96 well tissue culture plates (FIG. 6). Cells were seeded at a density of 10.sup.4 cells/cm.sup.216 hours before treatment in RPMI1640 containing 1% FCS (low concentration of additional growth factors). Cells were pre-treated with 100 nM anti-HER2 agents for 2 hours. Afterwards, cells were stimulated by adding heregulin beta-1 (HRG) to a concentration of 1 nM (Recombinant Human NRG1-1/HRG1-1: 26.9 kDa). HRG treatment leads to an increase of viable BT474 cells by 20%, compared to the control growth in 1% FCS alone (FIG. 6). The single treatments with anti-HER2 agents are thus compared to the corresponding controls in the absence (100% viability) or presence (120% viability) of HRG. Trastuzumab (hu4D5) treatment reduced viability by 50-60% in the absence of HRG, but did not show anti-tumor activity in ligand-stimulated cancer cells. Trastuzumab completely looses anti-tumor activity in presence of 1 nM HRG. Pertuzumab (hu2C4) treatment reduced viability by 20-30% in the presence or absence of HRG. Bispecific targeting agents reduced the viability by 80-90% in the absence of HRG and also showed 40-50% reduction in the presence of HRG. Therefore, the bispecific targeting agents show strongest anti-tumor activity both in the presence and in the absence of HRG. The additive effect of trastuzumab and pertuzumab resembles the individual maximal anti-tumor activity of the single agents (data not shown), but has no significant mechanistically synergism in in-vitro models. Therefore, the mechanism of action of bispecific targeting agents is superior to the treatment with trastuzumab combined with pertuzumab in in-vitro models. The treatment with the bispecific reagents exceeds the effect of the sum of effects from both antibodies.

(32) A person skilled in the art will appreciate that the XTT-assay is a suitable test for the determination of the cytotoxicity and for the evaluation of the potential of anti-tumor candidate compounds (see Jost et al, (1992) Journal of Immunological Method, 147, 153-165; Scudiero et al. (1988) Cancer Research, 48, 4827-4833; Andjilani et al, (2005) Int. J. Cancer, 117, 68-81; Rubinstein et al. (1990) J Natl Cancer Inst, 82(13), 1113-1117; Monks et al. (1991) J Natl Cancer Inst, 83(11), 757-766).

Example 2: Construction Plan of Bispecific Anti-HER2 Targeting Agents that Induce Apoptosis in HER2 Dependent Cancer Cells

(33) Generation of Binding Agents that Form the Components of the Active Molecule

(34) Binding molecules were obtained by ribosome display selection of ankyrin repeat protein libraries for specific binding to the full length extracellular domain of HER2 (ECD HER2) by methods previously disclosed (Zahnd et al. (2006) J. Biol. Chem. 279, 18870-18877).

(35) Preparation of the Biotinylated HER2 Target

(36) In order to obtain binders to the individual domains, the different individual domains of HER2 were individually expressed in insect cells, using a baculovirus expression system. Thereby, it is guaranteed that binders selected will be directed towards the domain of interest. Briefly, recombinant ErbB2-ectodomains carrying an N-terminal melittin signal sequence (MKFLVNVALVFMVVYISYIYA, SEQ ID 101) and an N-terminal His6 tag were expressed in Spodoptera frugiperda (Sf9) cells using baculoviral vectors. Sf9 cells were grown to a density of 410.sup.6 cells/mL and co-infected with the respective virus at a MOI of 1. 72 h post-infection, cells were harvested by centrifugation (30 min, 5,000 g, 4 C.) and the cleared medium was subjected to immobilized metal ion affinity chromatography (IMAC) purification with Ni-NTA Superflow purification resin.

(37) To generate binders against any domain of the extracellular region, the extracellular domain (residues 1-621) of HER2 was used as target for the selection with ribosome display (Zahnd et al., J. Biol. Chem. (2006) 281: 35167-35175) or, to generate binders against the first three domains, HER2 residues 1-509 was used.

(38) For immobilization, aliquots of these target proteins (200-600 g) were chemically biotinylated using EZ-Link Sulfo-NHS-SS-Biotin. Due to the size difference of the target proteins, a variable molar excess of the biotinylating reagent relative to the target protein was used (6-fold for HER2 1-621 or 1-509, 3-fold for the single domains). Reaction conditions were used according to the supplier's manual. Successful biotinylation was confirmed by ELISA and Western blot experiments. The biotinylated HER2 constructs were dialyzed extensively against PBS150.

(39) Target proteins had to be immobilized for selection. To avoid partial protein denaturation of the target proteins that may result from direct immobilization on solid plastic (i.e. polystyrene) surfaces, biotinylated target proteins were bound to neutravidin or streptavidin, which had been immobilized directly on a solid plastic surface, as follows: neutravidin (66 nM, 100 l/well) or streptavidin (66 nM, 100 l/well) in PBS was immobilized on MaxiSorp plates (Nunc, Denmark) by incubation at 4 C. overnight. The wells were blocked with 300 l of PBSTB (PBS containing 0.1% Tween-20, 0.2% BSA) for 1 h at room temperature. Binding of the biotinylated target proteins (100 l, 100 nM for selection) in PBSTB was allowed to occur for 1 h at 4 C. For the first selection round on immobilized target protein, requiring larger volumes, neutravidin (66 nM, 4 ml/tube) in PBS was immobilized on MaxiSorp Immunotubes by incubation at 4 C. overnight. The tubes were blocked with 4 ml of PBSTB for 1 h at room temperature. Binding of the biotinylated target proteins (4 ml, 100 nM) in PBSTB was allowed to occur for 1 h at 4 C. For selection on immobilized target protein, neutravidin and streptavidin were used alternately in selection rounds to avoid selection of binders against these proteins.

(40) Ribosome Display

(41) Ribosome display followed the published protocols (Dreier et al. (2012) Methods Mol. Biol. 805, 261-286; Zahnd et al. (2007) Nat. Methods 4, 269-279.) Typically 3 or 4 rounds were carried out. The first round was always carried out on plates, the later rounds in some of the selection on plates, in others in solution, where the biotinylated HER2 target is then bound to streptavidin-coated magnetic beads, as described in the protocols in detail (Dreier et al. (2012) Methods Mol. Biol. 805, 261-286; Zahnd et al. (2007) Nat. Methods 4, 269-279.).

(42) In the forth round, the selection pressure was increased by applying off-rate selection. For this purpose, after the in vitro translation was stopped by 5-fold dilution into ice-cold WBT buffer (50 mM Tris acetate, pH 7.5, 150 mM NaCl, 50 mM Mg(CH3COO.sup.).sub.2, 0.05% Tween 20), biotinylated HER2 construct was added to a final concentration of 10 nM, and the translation was allowed to equilibrate for 2 h at 4 C. The translation reaction was split into two aliquots, and non-biotinylated HER2 construct was added to a final concentration of 1 M to each aliquot, corresponding to a 100-fold excess over biotinylated antigen. The aliquots were incubated for 2 and 20 h, respectively, to increase the selection stringency for slower off rates. Ribosomal complexes were recovered using 30 l of streptavidin-coated magnetic beads. In a subsequent round, 175 nM biotinylated HER2 construct was immobilized on a NeutrAvidin-coated Maxisorp plate, i.e. rather non-stringent conditions to collect the binder (collection round) (Dreier et al. (2012) Methods Mol. Biol. 805, 261-286; Zahnd et al. (2007) Nat. Methods 4, 269-279.)

(43) In all selection rounds on solid-phase immobilized HER2 construct, a prepanning step of 30 min on a neutravidin-coated Maxisorp plate was performed as described (Dreier et al. (2012) Methods Mol. Biol. 805, 261-286; Zahnd et al. (2007) Nat. Methods 4, 269-279.). After prepanning, the translation extracts were allowed to bind for 45 min to HER2 construct-coated Maxisorp plates. Retained complexes were extensively washed with WBT buffer.

(44) Phage Display

(45) Phage display of the DARPin library followed the published protocol (Steiner et al. (2008) J. Mol. Biol. 382, 1211-1227). The immobilization of the various biotinylated HER2 constructs has been described above.

(46) Unless stated otherwise, all steps of the phage display selection were carried out at room temperature. Selection rounds were performed either on biotinylated target protein in solution with subsequent capturing on streptavidin-coated magnetic beads (referred to as: target protein in solution) or on biotinylated target protein bound to neutravidin or streptavidin, which had been directly immobilized on a solid plastic surface (referred to as: immobilized target protein), as described below. Very good results were obtained when performing the first selection round of selection on immobilized target protein, presumably because of the greater efficiency of capturing binders (especially important in the first round), followed by further rounds on target protein in solution, presumably because of the lower enrichment of background binders

(47) Selection on Target Proteins in Solution

(48) When the first selection cycle was done in solution, about 2.510.sup.13 phage particles of the phage DARPin library were incubated for 1 hour with 100 nM biotinylated target protein in 2 ml PBSTB for the first round of selection. In subsequent selection rounds, about 10.sup.12 phage particles were used (see below). The phage-antigen complexes were then captured on 100 l streptavidin-coated paramagnetic beads (10 mg/ml) for 20 min. After washing the beads eight times with PBST (PBS, 0.1% Tween-20) the phage particles were eluted with 200 l of 100 mM triethylamine (Et3N, pH not adjusted) for 6 min, followed by 200 l of 100 mM glycine-HCl, pH 2, for 10 min. Eluates were neutralized with 100 l of 1 M Tris-HCl, pH 7, or 18 l of 2 M Tris-base, respectively, combined and used to infect 5 ml of exponentially growing E. coli XL1-Blue cells. After shaking for 1 hour at 37 C., cells were expanded into 50 ml of fresh 2YT medium (5 g NaCl, 10 g yeast extract, 16 g tryptone per liter) containing 10 g/ml cam and incubated at 37 C. with shaking. After a maximum of 5 h (shorter times if OD.sub.600=0.5 was reached earlier), isopropyl--D-thiogalactoside (IPTG) was added to a final concentration of 0.2 mM and 15 minutes later the phage library was rescued by infection with VCS M13 helper phage at 10.sup.10 pfu (plaque forming units) per ml (multiplicity of infection 20). Cells were grown overnight at 37 C. without the addition of kanamycin. Cells were removed by centrifugation (5600 g, 4 C., 10 min) and 40 ml of the culture supernatant was incubated on ice for 1 hour with one-fourth volume of ice-cold PEG/NaCl solution (20% polyethylene glycol (PEG) 6000, 2.5 M NaCl). The precipitated phage particles were then collected by centrifugation (5600 g, 4 C., 15 min) and redissolved in 2 ml of PBS and used for the second round of selection.

(49) For the subsequent selection rounds, about 10.sup.12 of the amplified phage particles were used as input and incubated with 100 l of streptavidin-coated paramagnetic beads for 1 h to remove unspecific and streptavidin binding phage particles. After removing the beads, phage particles were incubated for 1 hour with 100 nM biotinylated target protein, complexes were captured on fresh beads, beads were washed 12 times with PBST, phages eluted with 400 l of 100 mM glycine-HCl, pH 2, for 10 min, the eluate neutralized with 36 l of 2 M Tris-base and phage particles amplified and purified as described above.

(50) After three rounds, enrichment of phage particles displaying DARPins binding specifically to the HER2 target construct was monitored by phage ELISA. About 510.sup.10 phage particles (estimated spectrophotometrically) of the initial library and the amplified pools of each selection round were pipetted to wells with and without immobilized target protein and incubated at RT for 2 h. After washing the wells four times with 300 l of PBST, bound phage particles were detected with mouse anti-M13 antibody horseradish peroxidase conjugate and soluble BM Blue peroxidase (POD) substrate.

(51) Selection on Immobilized Target Proteins

(52) For the first selection cycle about 3.510.sup.13 phage particles of the phage DARPin library were added to an immunotube containing the immobilized target protein (biotinylated target protein bound to neutravidin, which had been directly immobilized on the solid plastic surface) and incubated with rotation for 2 h. After rinsing the tube ten times with PBST, the phage particles were eluted with 500 l of 100 mM Et.sub.3N (pH not adjusted) for 6 min, followed by 500 l of 100 mM glycine-HCl, pH 2, for 10 min. Eluates were neutralized with 250 l of 1 M Tris-HCl, pH 7, or 45 l of 2 M Tris-base, respectively, combined and used to infect 13 ml of exponentially growing E. coli XL1-Blue cells. After shaking for 1 hour at 37 C. cells were expanded into 130 ml of fresh 2YT medium containing 10 g/ml chloramphenicol (cam) and incubated at 37 C. with shaking. Phage amplification and precipitation was done as described above.

(53) In the subsequent selection rounds about 10.sup.12 of the amplified phage particles were first incubated in a blocked immunotube (coated either with neutravidin or streptavidin used for immobilization of the target protein in the previous round of selection and BSA) one hour to remove neutravidin, streptavidin or unspecific binding phage particles. For the binding selection the phage particles were incubated for one hour in four wells containing the immobilized biotinylated target protein (directly coated neutravidin or streptavidin were alternately used in subsequent selection rounds). The wells were washed 12 times with PBST, phages eluted from each well with 100 l of 100 mM glycine-HCl, pH 2, for 10 min, the combined eluates neutralized with 36 l of 2 M Tris-base and phage particles amplified and purified as described above. After three rounds, enrichment was determined by phage ELISA as described above.

(54) Phage Display from Antibody Library

(55) Single-chain antibody fragments (scFv) were selected for binding to HER2, which have a molecular weight of 30 kDa, from HuCAL-1 (Knappik et al., 2000), a library of synthetic human antibody fragments. The library has a diversity of about 210.sup.9 members (Knappik et al., JMB, 2000, 296(1), 57-86). M13 phages presenting the HuCAL-1 scFv library as a fusion to the CT domain of g3p coat protein were selected for binding to soluble biotinylated HER2 domain 1 or domain 4, which was immobilized on neutravidin or streptavidin on microtiter wells as described above.

(56) Phage selections were performed by incubating 50 pmol of biotinylated antigen with 1 pmol of phages in 100 l PBS 0.5% BSA for 1 h at 4 C. The complexes were captured with 1 mg of BSA-blocked streptavidin magnetic particles and washed 10 times with PBS 0.5% BSA. Bound phages were eluted with 100 mM glycine, pH 2.2, and neutralized with the same volume of 1 M Tris, pH 8. E. coli TG1 cells were infected with eluted phages and plated on LB agar plates containing 1% glucose and 34 mg/l chloramphenicol. The plates were incubated overnight at 30 C., and bacteria were scraped off to inoculate 2YT medium containing 1% glucose and 34 mg/l chloramphenicol. The culture was incubated at 37 C. and at OD.sub.600=0.5 the phage library was rescued by infection with VCS M13 helper phage (Stratagene). The bacteria were harvested by centrifugation and resuspended in 2YT medium containing 30 mg/l kanamycin, 34 mg/l chloramphenicol, 0.1 mM IPTG and grown overnight at 30 C. Phages were precipitated from the culture supernatant by addition of polyethylene glycol PEG-6000 (3.3% final concentration), NaCl (0.4 M final concentration). Phages were resuspended in H.sub.2O, precipitated by addition of polyethylene glycol PEG-6000 (3.3% final concentration), NaCl (0.4 M final concentration) and resuspended in PBS.

(57) After the fourth and fifth round of phage display, pools of selected scFv-encoding sequences were subcloned via restriction sites XbaI and EcoRI into the expression plasmid pMX7 (Knappik et al., JMB, 2000, 296(1), 57-86). E. coli SB536 cells were transformed with the constructed vector. Bacteria were grown at 37 C. in 2YT medium containing 0.1% glucose and 34 mg/l chloramphenicol. At OD.sub.600=0.5 cultures were induced with 1 mM IPTG. ScFv fragments are secreted to the periplasm of E. coli. For small-scale expressions, cultures were incubated for 5 h after induction at 30 C. For periplasmic extracts, cells were collected by centrifugation and incubated overnight in 300 mM boric acid, 150 mM NaCl, 2 mM EDTA, pH 8, at 4 C. After centrifugation, the supernatant was used for enzyme linked immuno-sorbent assay (ELISA) screening.

(58) For large-scale expression of scFv fragments, cultures were incubated for 20 h at 22 C. Bacteria were collected by centrifugation and resuspended in 50 mM NaH.sub.2PO.sub.4, 300 mM NaCl, pH 8. After addition of a spatula tip of DNAseI and 2 mM MgCl.sub.2, bacteria were lysed in a French pressure cell. The lysate was filtered and purified on Ni-NTA agarose, washing with 16 column volumes of 50 mM NaH.sub.2PO.sub.4, 300 mM NaCl, pH 8; 12 column volumes of 50 mM NaH.sub.2PO.sub.4, 900 mM NaCl, pH 8; 16 column volumes of 50 mM NaH.sub.2PO.sub.4, 300 mM NaCl, 0.1% Triton X-100, pH 8; and 8 column volumes of 50 mM NaH.sub.2PO.sub.4, 300 mM NaCl, pH 8. Eluates were concentrated by ultra-centrifugation and buffer-exchanged to PBS using Micro BioSpin P-6 columns. For proliferation assays, samples were additionally purified on Detoxi-Gel endotoxin removal columns and eluted with PBS. When stored at 4 C. under sterile conditions, purified scFv fragments maintained unchanged binding activity for more than 3 months.

(59) Bispecific scFv1-Linker-scFv2 Constructs

(60) Antibody scFv fragments binding to either HER2 domain 1 or HER2 domain 4 were identified by ELISA as described above. From these scFv fragments, a series of bispecific scFv1-linker-scFv2 constructs (bispecific tandem scFv), where always a HER2 domain 1 binder was connected to a HER2 domain 4 binder (in either orientation), was constructed as follows: Since all HuCAL scFv fragments have common internal restriction sites, a vector could be constructed, pHu202, in which the upstream scFv fragment is connected via a flexible linker to the downstream fragment, which does not have a signal sequence, resulting in the arrangement phoA-scFv1-linker-scFv2, where phoA is the secretion signal. The linker segment can be exchanged via unique restriction sites that have been engineered into this fragment at its flanks, NotI and SfiI. Thus, all combinations of potential active bispecific antibodies were conveniently constructed by ligating the linker-scFv2 unit into the secretion vector containing phoA-scFv1, downstream of scFv1. After the active combinations had been identified, the linker was systematically varied in these constructs, by exchanging it into a series of linkers with different length, ligating it via NotI and SfiI.

(61) For large-scale expression of the scFv1-linker-scFv2 fragments, cultures were incubated for 20 h at 22 C. Bacteria were collected by centrifugation and resuspended in 50 mM NaH.sub.2PO.sub.4, 300 mM NaCl, pH 8. After addition of a spatula tip of DNAseI and 2 mM MgCl.sub.2, bacteria were lysed in a French pressure cell. The lysate was filtered and purified on Ni-NTA agarose, washing with 16 column volumes of 50 mM NaH.sub.2PO.sub.4, 300 mM NaCl, pH 8; 12 column volumes of 50 mM NaH.sub.2PO.sub.4, 900 mM NaCl, pH 8; 16 column volumes of 50 mM NaH.sub.2PO.sub.4, 300 mM NaCl, 0.1% Triton X-100, pH 8; and 8 column volumes of 50 mM NaH.sub.2PO.sub.4, 300 mM NaCl, pH 8. Eluates were concentrated by ultra-centrifugation and buffer-exchanged to PBS using Micro BioSpin P-6 columns. For proliferation assays, samples were additionally purified on Detoxi-Gel endotoxin removal columns and eluted with PBS.

(62) Bispecific Diabodies

(63) The cloning of the bispecific diabodies is similar to that of tandem scFvs, but with some important differences. We needed to clone two genes, phoA-VH1-VL2, followed by phoA-VH2-VL1. For simplicity, we opted for two promoters, each driving one of the genes. VH1 and VL1 are the heavy and light chain variable regions of svFv1, and VH2 and VL2 correspondingly of svFv2, but in the diabody arrangement they are now connected to the partner chain of the other scFv. The modularity of the synthetic HuCAL library with its conserved restriction sites within the synthetic genes makes this cloning very convenient. As can be seen, it was only necessary to exchange VH (or VL) between to scFv fragments, using the unique restriction sites by which VH and VL are flanked in the scFv fragment (Knappik et al., 2000). The whole cassette, promoter-phoA-VH1-linker-VH2 had been flanked by Not1 and SfiI sites in the newly created vectors pDia202, while in pDia203, the same sites had been engineered downstream of the scFv expression cassette. Thus, the complete unit promoter-phoA-VH1-linker-VH2 could be cloned into a vector already containing promoter-phoA-VH2-linker-VH1. Thus, both chains of the diabody were encoded on the same plasmid. Both are secreted to the periplasm where they assemble.

(64) For large-scale expression of the diabodies, cultures were incubated for 20 h at 22 C. Bacteria were collected by centrifugation and resuspended in 50 mM NaH.sub.2PO.sub.4, 300 mM NaCl, pH 8. After addition of a spatula tip of DNAseI and 2 mM MgCl.sub.2, bacteria were lysed in a French pressure cell. The lysate was filtered and purified on Ni-NTA agarose, washing with 16 column volumes of 50 mM NaH.sub.2PO.sub.4, 300 mM NaCl, pH 8; 12 column volumes of 50 mM NaH.sub.2PO.sub.4, 900 mM NaCl, pH 8; 16 column volumes of 50 mM NaH.sub.2PO.sub.4, 300 mM NaCl, 0.1% Triton X-100, pH 8; and 8 column volumes of 50 mM NaH.sub.2PO.sub.4, 300 mM NaCl, pH 8. Eluates were concentrated by ultra-centrifugation and buffer-exchanged to PBS using Micro BioSpin P-6 columns. For proliferation assays, samples were additionally purified on Detoxi-Gel endotoxin removal columns and eluted with PBS.

(65) In addition, single-chain diabody constructs were constructed as described in Example 5, (analogous to constructs described by Vlkel et al. (2001), Protein Engineering 14, 815-823).

(66) Analysis of Single Binding Agents

(67) Binding agents were characterized by means of enzyme-linked immunosorbent assay (ELISA). ELISAs, using the full length extracellular domain of HER2 (ECD HER2) for coating, were carried out to show binding of all individual binding agents. ELISA, using a truncated form of ECD HER2 (domain 1-3) as target, were performed to show specific binding of the DARPins to this part of HER2 ECD. This was originally applied to the collection of the 9XX series of binders (molecules originating from the HER2_509 selection). Domain 4 binders G3 and H14 were identified by binding to full length ECD HER2 but an absence of binding to the truncated ECD HER2 comprising only domains 1 to 3.

(68) Specific binding experiments were carried out on the surface of viable HER2 overexpressing cancer cells e.g. BT474, SkBr3, SkOv3, using standard flow cytometry methods. Multiple fluorescent detection systems, like e.g. detection of the His-Tag by an anti His-tag antibody, followed by a secondary antibody labeled with Alexa488, or alternatively, genetic superfolder GFP (sfGFP) fusions with the binding molecules or using directly Alexa488-labeled binding reagents, were used to confirm specific binding of all single binding reagents to the surface of HER2 overexpressing cancer cells. The binding to a single epitope was confirmed by the analysis of mean fluorescence intensities, resulting in similar values for all binders at saturation, and more importantly, by complete inhibition of the signal when competed to an unlabeled control binding to said epitope. The single binding reagents also passed different quality control measurements like e.g. size exclusion chromatography, multi-angle light scattering and polyacrylamide gel electrophoresis (PAGE).

(69) Competitive Binding Analysis of Binding Reagents

(70) Competitive binding analysis was performed to characterize the epitopes of the binding agents of the 9XX collection. All binding agents of the 9XX collection compete for binding to a similar epitope on domain 1 of HER2, except binder 9.01. Competitive binding FACS analysis was also performed with domain 4 binding agents versus trastuzumab. Groups of competing and non-competing binding agents were identified. Importantly, binding to the trastuzumab epitope is not a prerequisite for the anti-tumor activity of the bispecific molecules (G3 does not compete with trastuzumab for binding). Binder H14 does compete with G3 and does show competition with trastuzumab.

(71) Competitive binding FACS analysis performed with the 9XX binding molecules versus pertuzumab binding did not show competition. None of the single binding agents binds to the pertuzumab epitope. ELISA, using the domain 1 of the ECD HER2 as target, was performed to show specific binding of the 9XX collection.

(72) Table 1 summarizes properties of preferential binding units (that can be components of bispecific molecules with bioactivity) and control binding units (which do not contribute bioactivity) for the construction of bispecific binding agents with superior anti-tumor activity. Listed are the single domains of the extracellular part of HER2 that are bound by the single agents. The epitope is characterized by inhibition of a binding assay performed in ELISA or on the surface of HER2 overexpressing cancer cells by means of flow cytometry. Crystal structure data are available for the indicated binding agents, which characterize the specific epitopes in detail on the single amino acid level. For the construction of potent bispecific anti-tumor agents, a binding agent which targets domain 1 of HER2 is preferentially fused to a binding agent that targets domain 4 of HER2 from the list of indicated binding agents.

(73) TABLE-US-00003 TABLE 1 Summary of single binding agents Strong Binds Competitive anti-tumor to Binding to Crystal activity in HER2 HER2 known Structure bispecific domain: with: available: setup: G3 IV H14 YES YES H14 IV G3; 4D5 YES 902 I 929; 926 YES 903 I 929; 926 YES 910 I 929; 926 YES 916 I 929; 926 YES 926 I 929; 926 YES YES 929 I 929; 926 YES YES 930 I 929; 926 YES H01 I 929; 926 YES H03 I 929; 926 YES Off7 none none YES 4D5, IV H14, YES YES trastuzumab Nanobody, Zybody 2C4, II Nanobody, YES pertuzumab Zybody zHER2 III none YES A21 I none YES YES

(74) The domain 1-binding scFv A21 is described in example 5.

(75) Expression of Bispecific Binding Agents

(76) The genes or coding sequences of the bispecific molecules were constructed in a vector pQiBi-01- (or -11-; -12-; -22-; -23-; -33-); using conventional restriction digest and ligation techniques with a BamHI/HindIII restriction site for the N-terminal binding molecules and BglII/BsaI restriction sites for the C-terminal binding molecules. This vector is derived from pQE30, but encodes the lacl.sup.q gene and unique restriction sites (BamHI/HindIII and BglII/BsaI, respectively) to clone one binder upstream, the other downstream of a linker via BamHI/HindIII. The numbers indicate the different linker lengths, where each unit is a (Gly.sub.4Ser, SEQ ID 51) unit. E.g., the pQiBi-22-vector encodes 4 (Gly.sub.4Ser) units (SEQ ID 54 between the binders.

(77) Bispecific constructs were expressed in E. coli strains XL1blue or E. coli BL21 using the lac-operon induction system by isopropyl--D-thiogalactopyranoside (IPTG). Bacteria were lysed by the French press method or by sonification. Filtered bacterial lysates were loaded on NiNTA-agarose bench top columns, washed with TBS_W (50 mM Tris, 400 mM NaCl, 20 mM imidazole, pH 7.5) and in addition washed with 70 CV PBS containing 0.1% Triton X-114 for endotoxin removal. Proteins were eluted in PBS containing 250 mM imidazole. Proteins were further purified by size exclusion chromatography using PBS buffer. Limulus amebocyte lysate (LAL)-assays were performed to assess endotoxin content. Protein concentrations were determined by absorbance spectroscopy at 280 nm and or by a BCA-assay.

(78) Further bispecific agents are described in Examples 5 and 6.

(79) Analysis of Bispecific Binding Reagents

(80) Bispecific binding reagents passed quality control measurements for molecular weight, monomeric status and binding to ECD HER2. Bispecific binding agents comprising trastuzumab-competing binders (in the example, DARPin H14) also compete with trastuzumab in the bispecific setup, as expected. Bispecific binders that do not contain a trastuzumab-competing unit did not show competition in the bispecific setup, also as expected. Competitive binding ELISA, using full length HER2 ECD as target, was performed with all bispecific binding agents also versus pertuzumab. None of the bispecific binding agents competes with pertuzumab for binding to full length ECD HER2 in ELISA. Binding to the surface of viable HER2 overexpressing cancer cells was shown by flow cytometry.

(81) For determination of the anti-tumor activity of the bispecific agents (FIG. 8), BT474 cells were seeded into 96 well plates 16 h before treatment at a density of 1010.sup.4 per cm.sup.2 in RPMI1640 containing 10% FCS. Titrations from 100 pM to 1 M of each agent (final concentrations) were added and cells were treated for 4 days in a cell culture incubator. XTT viability assays were used according to the manufacturer's protocol to assess the remaining viability of the cancer cells. The targeting agents can be grouped according to their anti-tumor activity. The single binding agents scFv 4D5 and DARPin H14 reduced the cell growth by a similar extent, by 20-30%. Trastuzumab reduced the cell growth by an extent of approx. 50%. The flexible bispecific agents 926-FL-G3 and 929-FL-H14 reduced the cell growth by a similar extent of 80-90%.

(82) All bispecific constructs that share a similar epitope with e.g. monovalent DARPin 929 on domain 1 of HER2 ECD show strong anti-tumor activity in cell proliferation assays (FIG. 9). BT474 cells were seeded into 96 well plates 16 h before treatment at a density of 10.sup.4 per cm.sup.2 in RPMI1640 containing 10% FCS. Anti-HER2 binding agents were added to a concentration of 100 nM (final concentration), and cells were treated for 4 days. XTT cell proliferation assays were developed according to the manufacturer's protocol. All bispecfic agents containing 9XX at the N-terminus, which showed competitive binding with 926 and 929 in ELISA to ECD HER2, reduced the viability of the cancer cells by 70-80%, i.e. to a higher extent than trastuzumab.

(83) For determination of the anti-tumor activity of single binding agents, BT474 cells were seeded into 96 well plates 16 h before treatment at a density of 10.sup.4 per cm.sup.2 in RPMI1640 containing 10% FCS. Anti-HER2 binding agents were added to a concentration of 100 nM, and cells were treated for 4 days. XTT cell proliferation assays were developed according to the manufacturer's protocol. H14, the HER2 domain 4 binding agents which competes for binding with trastuzumab (hu4D5), reduces tumor growth by 20%. The 9XX domain 1 binding agents do not show any anti-tumor activity as single binding agents (FIG. 10).

(84) The combination treatment of the single anti-HER2 binding agents is shown in FIG. 11. BT474 cells were seeded into 96 well plates 16 h before treatment at a density of 10.sup.4 per cm.sup.2 in RPMI1640 containing 10% FCS. Anti-HER2 binding agents were added to a concentration of 100 nM, and cells were treated for 4 days. XTT cell proliferation assays were developed according to the manufacturer's protocol. The 9XX domain 4 binding agents do not show an additive effect to anti-tumor activity of H14. Thus, the strong anti-tumor activity requires that the binding agents are connected into a bispecific molecule.

(85) Cell proliferation assays with trastuzumab-resistant cell lines are shown in FIG. 12. Cancer cells were seeded into 96 well plates 16 h before treatment. A serial dilution of anti-HER2 binding agents was added and cells were treated for 4 days. XTT cell proliferation assays were developed according to the manufacturer's protocol. The anti-tumor activity of bispecific targeting agents is similarly modest to trastuzumab in trastuzumab-resistant cell lines.

Example 3: Differentiation from Prior Art Constructs: Comparison of Apoptosis Induced by 7C2 in Combination with 4D5 Versus Bispecific Targeting Agents

(86) As was demonstrated in the patent (U.S. Pat. No. 7,371,376 B1; US20110033460 (A1) ANTI-ErbB2 ANTIBODIES), the antibody 7C2 is competent as a single agent to induce apoptosis in the following cell lines BT474, SkBr3, SkOv3 or Calu-3. The epitopes on domain 1 of the ECD HER2 bound by 7C2 and 7F3 are different from the epitopes bound by the 9XX collection (see below), and are also different from those of scFv fragment A21 (see example 5 below) The bispecific targeting agents disclosed here induce apoptosis in BT474 and SkBr3 cells, but not in SkOv3 cells. The absence of anti-tumor activity in SkOv3 cells can be explained by the activating mutation H1047R of the PI3-Kinase. The induction of apoptosis by the bispecific targeting agents is thus correlated with a non-mutated, wild-type downstream signaling pathway of HER2 and HER3.

(87) The absence of anti-tumor activity is another difference to the antibodies 7C2 and 7F3, which show anti-tumor activity as single agents. In US20110033460A1, an additive effect of 7C2 and 4D5 (trastuzumab) to anti-tumor activity is shown. In contrast, the anti-tumor activities of bispecific targeting agents disclosed here are significantly reduced in combination with trastuzumab (FIG. 13).

(88) Even more importantly, the monospecific, bivalent constructs made in analogy to the targeting agents disclosed here, are not active when mixed (FIG. 14; see detailed description of this experiment below). This is in contradistinction to the mixture of the antibody 7C2 with 4D5 and 7F3 with 4D5. This underlines that the mechanism of action of said antibody mixtures is completely different to the bispecific targeting reagents disclosed herein. For the bispecific targeting reagents disclosed herein, the covalent linking of a domain I binding unit to a domain IV binding unit is essential for the mode of action.

(89) In the case of H14 fusions this reduced activity can be explained by simple competition for binding to the same epitope, while in the case of G3 fusions, trastuzumab and G3 do not compete for binding to domain 4. Hence, trastuzumab blocks the formation of inactive HER2 homodimers that are induced by the bispecific molecules according to the invention. Therefore, the modes of action of 7C2 in combination with 4D5, in comparison to the bispecific targeting agents according to our invention, are different. Furthermore, the concept for induction of apoptosis in HER2 overexpressing cancer cells is completely different. Here it is shown that through the strong inhibition of the internal cell signalling in these HER2-dependent cancer cells, apoptosis is induced by the bispecific binding molecules. In contrast, 7C2, a homobivalent IgG, is shown to induce apoptosis but not inhibition of cell growth. This mode of action uncouples signalling from apoptosis and is therefore more similar to e.g. death receptor signaling (FAS or TNF receptor). The inventors believe, without wishing to be bound by theory, that the bispecific reagents according to the present invention work mainly by preventing formation of active dimers and act thus at the level of signaling. Downregulation of receptors is not likely to form an intrinsic part of the mechanism of the bispecific molecules disclosed here. In contrast, it may be part of the mechanism of action of the combination of 7C2 in combination with 4D5.

(90) The antibodies trastuzumab (TT, 4D5) and pertuzumab (PER, 2C4) disrupt the inactive HER2 homodimers formed by bispecific targeting agents (FIG. 13). BT474 cells were seeded into 96 well plates more than 16 h before treatment at a density of 10.sup.4 per cm.sup.2 in RPMI1640 containing 10% FCS. The bispecific targeting agents 926-G3 and 929-H14 were added at a concentration of 100 nM. Subsequently, titration from 10 pM to 1 M of an anti-HER2 antibody, either trastuzumab (TT, 4D5) or pertuzumab (PER, 2C4), was added. BT474 cells were treated for 4 days in a cell culture incubator at 37 C. and 5% CO.sub.2. XTT cell viability assays were performed according to manufacturer's protocol. The absorbance at 450 nm correlates with the number of viable cells. By increasing concentrations of trastuzumab or pertuzumab in the presence of the bispecific agents 926-G3 and 929-H14, the antitumor activity of the bispecific targeting agents is significantly reduced. This indicates that the anti-tumor effect of the bispecific molecules according to this invention is greater than that of trastuzumab or pertuzumab.

(91) The anti-tumor activity of bispecific targeting agents is not caused by random cross-linking of receptors (FIG. 14; Acontrol; C926-22-926/H14R-22-H14R; D926AvantE-22-926AventE/H14R-22-H14R; E926AvantE-22-926AventE/H14AvantE-22-H14AventE; F926-22-926/G3-22-G3; first column=10 pM, second column=100 pM, third column=1 nM, forth column=10 nM, fifth column=100 nM and sixth column=1 M of C, D, E and F, respectively). BT474 cells were seeded into 96 well plates more than 16 h before treatment at a density of 10.sup.4 per cm.sup.2 in RPMI1640 containing 10% FCS. Combinations of homo-bivalent targeting agents were titrated from 10 pM to 1 M. The combination of both homo-bivalent targeting agents did not show any signification reduction in the viability of the cancer cells.

(92) Bispecific targeting agents do not compete for binding with pertuzumab in ELISA (FIG. 15, A-pertuzumab, 2ndAb (no competitor), B-2nd Ab, C-pertuzumab, 2nd Ab (no ErbB2), D-2nd Ab (no ErbB2)). Wells of the MaxiSorp plate were coated with 100 l PBS containing 66 nM streptavidin for 12 hours at 4 C. Liquids were removed completely after each step. The plastic surface was blocked by PBS_TB (PBS containing 0.1% Tween20, 0.2% BSA) for 1 hour at room temperature with continuous shaking. Afterwards, 20 nM of truncated ErbB2-avidin conjugate was added in 100 l PBS_TB and incubated for 1 hour. The plate was washed four times with PBS_TB. Then, bivalent DARPins were added to 1 M in PBS_TB, and binding took place for 3 hours on a shaker. Next, 1 nM of pertuzumab was added and incubated for 30 min. The plate was washed four times in PBS_TB. The secondary anti-human antibody coupled to alkaline phosphatase was incubated in 100 l PBS_TB for 1 hour. The plate was washed four times with PBS_TB. Finally, 100 l of freshly prepared and filtered pNPP buffer (3 mM pNPP, 50 mM NaHCO.sub.3, 50 mM MgCl.sub.2) was added and the color reaction was developed for 5 min at room temperature. Absorbance was detected on an ELISA plate reader at the wavelength of 405 nm.

(93) Analysis of competitive binding to domain 4 of HER2 was measured by flow cytometry (FIG. 16). 10.sup.5 BT474 cells were incubated with either 1 M of G3 or H14 for 30 min at room in 100 l PBS_BA (PBS, 0.2% NaN.sub.3, 1% BSA). Subsequently, Alexa4.sub.88-trastuzumab, which had been labeled with Alexa.sub.488-succinimidyl ester, was added to a concentration of 100 nM and incubated for 30 min at room temperature. Afterwards, cells were washed twice using PBS_BA. Flow cytometry measurements were performed on a Cyflow space system. 10.sup.4 events were recorded in a FSC/SSC gate to measure cells with proper size. Mean fluorescence intensities were calculated by FlowJo software and data were normalized to the MFI of Alexa.sub.488-trastuzumab binding. G3 does not compete with the binding of trastuzumab, while H14 and trastuzumab bind to a very similar epitope and therefore show 100% competition for binding.

(94) Bivalent binding of the bispecific targeting agent to HER2 at the surface of cancer cells is a prerequisite for strong anti-tumor activity. To confirm the binding of bispecific agents, the association rate constant k.sub.on and dissociation rate constant k.sub.off on intact cells can be measured by flow cytometry (FIG. 17) (Tamaskovic et al. (2012) Methods Enzymol. 503, 101-134).

(95) The following tables show the determined binding affinities of single and bispecific binding agents and certain DARPins.

(96) TABLE-US-00004 average average average average k.sub.on (M.sup.1s.sup.1) k.sub.obs (s.sup.1) k.sub.off (s.sup.1) K.sub.d (M) 929 68977 0.0035 2.21 10.sup.3 33.47 10.sup.9 H14 196244 0.0037 1.79 10.sup.4 0.97 10.sup.9 929-FL-H14 77959 0.0015 3.99 10.sup.5 0.52 10.sup.9

(97) TABLE-US-00005 DARPin K.sub.D (nM) k.sub.on (10.sup.5 M.sup.1s.sup.1) k.sub.off (10.sup.3 s.sup.1) 916 (domain 1 binder) 6.9 1.2 0.9 926 (domain 1 binder) 1.4 0.7 0.1 929 (domain 1 binder) 3.8 2.0 0.8 H14 (domain 4 binder) 0.2 4.1 0.1

(98) Preparation of Cancer Cells for Flow Cytometry Measurements

(99) Cells were detached by collagenase and EDTA for 5 min at 37 C. The solution was quenched by addition of medium and centrifuged at 300 g for 3 min. Cells were washed twice in warm PBS. Cell densities were determined with a CASY cell analyzer and adjusted to 10.sup.6 cells per sample. Internalization was blocked by incubation in PBS containing 0.2% NaN.sub.3 and 1% BSA for 30 min at 37 C.

(100) Flow Cytometry Measurements

(101) Samples were resuspended in 1 ml cold PBS and measured on flow cytometer. 10,000 cells per sample were recorded. Results were gated for FSC vs SSC of the cells. Green fluorescence was detected with the FL1 detector. Data were processed by the FlowJo 7.2.5 software.

(102) Measuring Association of Binding Agents on the Surface of Cancer Cells

(103) For on-rate determinations, BT474 cells are incubated at a concentration of 110.sup.6 cells/ml with 2.5, 7.5, and 22.5 nM DARPin-Alexa Fluor-488 conjugates in PBSBA at room temperature for defined time intervals, ranging from 1 to 60 min. For each time point, a 1 ml aliquot of cells is withdrawn and subjected to FACS. Since the applied concentrations of the labeled ligand conjugates are very low, and since the time resolution of the measurement is to be maintained to ensure the accuracy of the on-rate determination, the samples are processed without further washing. For each time point, at least 10.sup.4 intact cells (gated as a uniform population on a FSC/SSC scatter plot) are counted, and the MFI (mean fluorescence intensity) is recorded.

(104) Measuring Dissociation of Binding Agents on the Surface of Cancer Cells

(105) 10.sup.6 cells per time point were incubated with 1 M Alexa488 labeled binding agents in 100 l PBS (0.2% NaN.sub.3, 1% BSA) for 1 hour at 4 C. on the shaker. Corresponding to 100 l cell suspension, samples were washed twice in 1 ml PBS (0.2% NaN.sub.3, 1% BSA) and centrifuged at 600 g for 30 sec at room temperature. Cells were resuspended in 1 ml PBS (0.2% NaN.sub.3, 1% BSA) containing 100 nM of equivalent unlabeled binding agent. The dissociation reaction was incubated for the indicated times (15, 30, 60, 120, 180 and 240 min) at room temperature while continuously stirring in the dark. Dissociation was stopped by placing the cell pellets on ice. Each sample was washed once with 1 ml cold PBS.

Example 4: Additional Data Regarding Construction and the Effects of Mono- and Bivalent Constructs on Cell Proliferation and Cell Death

(106) DARPins that had been selected by phage display or ribosome display to target the full-length ectodomain of HER2 without showing any cross-specificity against other EGFR-family members were characterized concerning which of the four HER2-subdomains forms the epitope. Since DARPins typically recognize conformational epitopes, subdomains were expressed alone and in combination in insect cells using a baculovirus system. To minimize glycosylation for subsequent crystallization, the Asn residues were replaced in predicted N-linked glycosylation sites by Asp. ELISAs on these proteins showed that the epitopes recognized by DARPins 9_26 and 9_29 are located on HER2-I, while DARPin G3 bound to HER2-IV. Competition for binding to HER2-overexpressing cells measured by flow cytometry revealed that DARPins 9_26 and 9_29 compete for the same epitope. DARPin G3, which binds to HER2 subdomain IV, did not compete with trastuzumab but competed with a different HER2-specific DARPin, H.14, which in turn competed with trastuzumab.

(107) Various bivalent and bispecific constructs were generated by genetically fusing two DARPins by (G.sub.4S).sub.n linkers of different lengths. To target two non-overlapping epitopes with a single molecule, DARPins 9_29 or 9_26 were connected to DARPin G3 by a 20 amino acid linker, with either an ECD-I binder at the N-terminal end and the ECD-IV binder at the C-terminus or in opposite orientation. The four different bispecific binders (e.g., 9_26-(G.sub.4S).sub.4-G3, abbreviated 6_20_G for the two DARPins and the linker length of 20 amino acids) were tested regarding their binding to HER2-overexpressing cells. G3 with a KD of 90 pM has the highest affinity of the three HER2-binders used in this study, compared to a KD of 1 nM for 9_26 and 1 nM for 9_29. Kinetic experiments on cells in the presence of a competing DARPin (to prevent rebinding) revealed that the off-rates of the bispecific binders were 10 times lower than the off-rates of monovalent G3 (FIG. 18A). The slower off-rate and higher KD of the bispecific constructs, compared to their monovalent building blocks, can be attributed to an avidity effect and indicates bispecific binding to HER2 on the cell.

(108) The influence of the different DARPin constructs on cell proliferation and cell survival were tested in XTT assays, using BT474 cells as an example of a HER2-addicted cell line. MCF7-cells, which express HER2 at much lower levels than BT474 cells, were used as a control. Calibration experiments showed that a signal decrease by 60%, compared to untreated cells, corresponded to lack of cell proliferation over the 4 days of cell growth before the XTT assaya larger decrease indicated cell death. The XTT assay were performed as described in example 1.

(109) None of the characterized monovalent DARPins affected the number of viable cells measured by the XTT assay (FIG. 18B). Mixtures of two different DARPins proved to be equally inert, as did control constructs in which one of the two DARPins in the bispecific molecule had been replaced by a non-HER2-binding DARPin (DARPin off7, targeting maltose-binding-protein) (FIG. 18C). A monospecific bivalent DARPin G_20_G even stimulated cell proliferation (FIG. 18C).

(110) Bispecific constructs composed of a subdomain I binder at the N- and the subdomain IV binder at the C-terminus (6_20_G or 9_20_G) showed a concentration-dependent decrease of cell viability by up to 75%, while treatment with trastuzumab decreased viability by 50% (FIG. 18D). The constructs with reverse orientation (G_20_9) either lacked any effect on cell-growth (G_20_6) or even slightly promoted cell growth. Similar to trastuzumab, bispecific constructs did not affect the cell-proliferation of MCF7-cells (FIG. 18E), suggesting the restriction of the observed effects to HER2-addicted cells. Comparison of constructs with 5, 10, 20, 30 and 40 amino acid linkers showed that for 9_x_G constructs, specific activity and potency decreases with increasing linker length. The most potent constructs proved to be 6_5_G and 9_5_G, with (G.sub.4S)-linkers of only five amino acids. They decreased the cell viability in XTT-assays after four days of growth by more than 80% as compared to untreated cells, and showed a half-maximal effect already at a concentration of less than 100 pM compared to ca. 1 nM for 6_20_G and 9_20_G. Conversely, increasing the linker length to forty amino acids, as in 6_40_G and 9_40_G, decreased the biological activity (growth reduction of only 40%) (FIG. 18F). The constructs with inverse orientation, G_x_6 and G_x_9, inactive or even stimulatory at a linker length of 20 amino acids, gained anti-proliferative activity at short linker lengths, but the best construct was found to be only as active as trastuzumab (FIG. 18G).

(111) Neither the single DARPins nor the bispecific constructs affected internalization or degradation of HER2, as determined by flow cytometry.

Example 5: Bispecific HER2 Bindings Agent with One or Two Antibody Fragments

(112) To demonstrate the cytotoxic activity of bispecific HER2 binding agents constructed from antibody fragments, bispecific constructs of the type scFv1-linker-scFv2; DARPin-linker-scFv; and scFv-linker-DARPin constructs were constructed. Here, in each fusion protein, one of the units (scFv1, scFv2, scFv or DARPin) binds to domain 1, the other one binds to domain 4.

(113) For a description of scFv1-linker-scFv2 constructs, cf. p. 37.

(114) To generate a domain 1-binding scFv, the scFv chA21 (A21) was used described in Hu S. et al., (2008) Proteins 70:938-949.). The crystal structure in complex with HER2 had been determined, verifying the binding of this scFv to domain 1. The protein sequence of the heavy and light chain of the scFv A21 was obtained from the PDB file (PDB ID: 2GJJ). A flexible glycine serine linker of 4GGGGS units (GGGGS GGGGS GGGGS GGGGS, SEQ ID 54) was introduced to connect the heavy and the light chain in either orientation: Two orientations were thus obtained, by either fusing the N-terminal heavy chain to the light chain (A21HL, SEQ ID 65) or the N-terminal light chain to the heavy chain (A21LH, SEQ ID 66 or SEQ ID 93) within one single protein sequence connected by the said glycine-serine linker.

(115) The respective gene sequences were synthesized by Genescript Inc., and they additionally contain a BamHI/HindIII cloning site for directional cloning (see below).

(116) To generate a domain 4-binding scFv, the scFv of the antibody hu4D5 was constructed. The crystal structure of the corresponding Fab fragment (hu4D5, trastuzumab; Herceptin) in complex with HER2 had been determined, verifying the binding of this scFv to domain 4, as described in Cho et al., (2003) Nature 421:756-760. The protein sequence of the heavy and light chain for the construction of the scFv 4D5 was obtained from the PDB file (PDB ID: 1N8Z). A flexible glycine serine linker of 4GGGGS units (GGGGS GGGGS GGGGS GGGGS, SEQ ID 54) was introduced to connect the heavy and the light chain in either orientation: Two orientations were thus obtained, by either fusing the N-terminal heavy chain to the light chain (4D5HL, SEQ ID 67) or the N-terminal light chain to the heavy chain (4D5LH, SEQ ID 68 or SEQ ID 92) within one single protein sequence connected by the said glycine-serine linker. Also, an additional scFv 4D5LH (SEQ ID 69) with an alternative has been created.

(117) The respective gene sequences were synthesized by Genescript Inc., and they additionally contain a BamHI/HindIII cloning site for directional cloning (see below).

(118) Construction of scFv1-Linker-scFv2; DARPin-Linker-scFv; and scFv-Linker-DARPin Fusion Proteins

(119) For the gene construction of bispecific fusions proteins, which contain a HER2 domain 1 and a domain 4 binding moiety, a generic vector (pMxAC) was used. This vector is based on pMx9 (Rauchenberger, R. et al. (2003) J. Biol. Chem. 278, 38194-38205), and contains an OmpA signal sequence for periplasmic expression in E. coli. The OmpA signal sequence was exchanged by a DsbA signal sequence taken from the vector pDSt066 (see description in Steiner et al. (2008) J. Mol. Biol., 382:1211-1127). In addition, a new multiple cloning site was introduced into the vector pMx9 containing the DsbA signal sequence, in which restriction sites allowed specific cloning on either side of the flexible gly-ser linker. These cloning cassettes therefore allowed the preparation of fusion proteins with different lengths of linkers originating from the plasmid pQiBi-22- (4GGGGs flexible linker, SEQ ID 54); pQiBi-11- (2GGGGs flexible linker, SEQ ID 52) and pQiBi-01- (1GGGGs flexible linker, SEQ ID 51) (Boersma et al. (2011), J. Biol. Chem. 286, 41273-41285.)

(120) The new vectors were termed pMxAC-22- (4GGGGs flexible linker, SEQ ID 54); pMxAC-11-(2GGGGs flexible linker, SEQ ID 52) or pMxAC-01- (1GGGGs flexible linker SEQ ID 51) respectively.

(121) These pMxAC vectors contain a BamHI/HindIII cloning site for inserting the N-terminal binding construct (upstream of the linker) and a BglII/BsaI site (compatible with BamHI/HindIII cloning sites) cloning site for introducing the C-terminal binding moiety (downstream of the linker). In addition, the construct contains a C-terminal 6His-tag for purification and detection and a FLAG-tag M1 for detection of periplasmic export (Knappik et al. (1994) Biotechniques 17, 754-761.).

(122) Map of the ORF in the pMxAC-22-Vector

(123) TABLE-US-00006 embedded image

(124) Legend, Explaining the Different Elements:

(125) TABLE-US-00007 MKKIWLALAGLVLAFSASA(SEQID112):DsbA-signal sequence,whichgetscleavedoff DYKDDI(SEQID113):FLAG-TagM1 GS:BamHIcloningsite embedded image KL:HindIIIcloningsite GGGGSGGGGSGGGGSGGGGS:Flexiblelinker(-22-/FL4, SEQID54) RS:BgIIIcloningsite embedded image KL:Bsa1cloningsite GS:flexiblespacer HHHHHH:6xHis-Tag(SEQID114)

(126) Alternative Vectors for scFv/DARPin Fusion Proteins

(127) In addition to the periplasmic expression in E. coli described above, expression of the scFv/DARPin fusion proteins was performed by secretion from Spodoptera frugiperda (Sf9) cells using the Multibac system as described previously (Fitzgerald et al. (2006) Nature Methods 3:1021-32.). In brief, the coding sequences of the fusion proteins were subcloned via ligation-independent cloning (LIC) into the donor vector pFLmLIC introducing an N-terminal melittin signal sequence (SEQ ID 99). The donor vectors were used to introduce the fusion protein coding sequences into the bacmid EmBacY. Baculoviruses for infection of Sf9 cells were generated through transfection of the bacmid DNA into Sf9 cells. For expression, Sf9 cells were grown to a density of 410.sup.6 cells/mL and co-infected with the respective virus at a MOI of 1. 72 h post infection, cells were harvested by centrifugation (30 min, 5000 g, 4 C.) and the cleared medium was subjected to immobilized metal ion affinity chromatography (IMAC) purification with Ni-NTA Superflow (Qiagen) purification resin.

(128) The following table shows the scFv/DARPin fusion proteins which were expressed in Sf9 cells or in E. coli. Note that the N-terminal melittin signal sequence (MVVYISYIY, SEQ ID 99) is cleaved upon protein secretion and not present in the secreted and purified proteins.

(129) TABLE-US-00008 scFv/DARPin fusion protein SEQ ID A21HL_L4_G3 70 A21LH_L4_G3 71 A21HL_L4_H14 72 H14_L4_A21LH 73 H14_L4_A21HL 74 G3_L4_A21LH 75 G3_L4_A21HL 76 A21HL_L1_G3 77 9.29_L1_4D5LH 78 926E-L4-4D5HL 88 926E-L4-4D5LH 89 929-L4-4D5HL 90 929-L4-4D5LH 91

(130) Expression scFv1-Linker-scFv2 Constructs in the Periplasm of E. coli

(131) ScFv1-linker-scFv2 constructs were co-expressed with periplasmic chaperones in the periplasm of E. coli. For this purpose, the pMxAC scFv1-linker-scFv2 plasmids were co-transformed with the plasmid pCH-A1 (Schaefer and Plckthun (2010) Improving expression of scFv fragments by co-expression of periplasmic chaperones, in: Antibody Engineering, Kontermann, and Dbel, eds., Vol. 2, 2nd edit., pp. 345-361, Springer Verlag, Berlin Heidelberg, Germany) into E. coli SF130 (Meerman and Georgiou (1994); Biotechnology (N Y) 12:1107-1110). After transformation, single clones of E. coli were adapted to Terrific Broth growth medium (TB; Cold Spring Harbor Protocols) overnight and transferred to 1 L TB expression culture to an initial OD.sub.600 of 0.1. ScFv fusion construct expression was induced by isopropyl--D-thiogalactopyranoside (IPTG), and expression was performed overnight at 25 C.

(132) Purification of scFv1-Linker-scFv2 Constructs from E. coli Expression Culture

(133) Expression cultures were pelleted by centrifugation, washed with Tris buffer (50 mM Tris base, 150 mM NaCl, pH 7.5) and resuspended in cold Tris buffer containing protease inhibitors (Rochecomplete protease inhibitor cocktail) and DNaseI (Roche) and kept at 4 C. during the entire process. E. coli were lysed with a French press and centrifuged for 30 min at 20,000 g. The supernatant was adjusted to a final concentration of 20 mM imidazole, 400 mM NaCl, 10% glycerol, pH 7.5, and applied to Ni-NTA bench-top columns. Columns were washed with 30 CV of Tris buffer containing 20 mM imidazole, 400 mM NaCl and 10% glycerol, high-salt washed with 30 CV Tris buffer containing 1 M NaCl, low-salt washed with 30 CV Tris buffer containing 10 mM NaCl. The bound fraction was eluted with Tris buffer containing 300 mM imidazole. Ni-NTA-eluted protein was loaded on a protein-A bench-top column, and endotoxin-washed with 80 CV phosphate buffer saline (Dulbecco's PBS) containing 0.1% Triton X-114, washed with 30 CV PBS and eluted with 4 CV 100 mM glycine buffer pH 3.6 into 4 CV of 1.5 M Tris buffer pH 8, 150 mM NaCl. Proteins were concentrated and dialyzed against HEPES buffer (25 mM HEPES, 150 mM NaCl, pH 7.5).

(134) The following table shows the scFv1-linker-scFv2 constructs that have been expressed in E. coli:

(135) TABLE-US-00009 scFv1-linker-scFv2 SEQ ID 4D5HL-L1-A21HL 80 4D5HL-L4-A21LH 81 4D5LH-L1-A21HL 82 4D5LH-L4-A21HL 83 4D5LH-L4-A21LH 84 A21HL-L4-4D5LH 85 A21LH-L1-4D5LH 86 A21LH-L4-4D5LH 87 4D5LH-L1-A21LH 100

(136) Diabody A21H_4D5LH_A21L

(137) The gene of the diabody construct (analogous to constructs described by Vlkel et al. (2001), Protein Engineering 14, 815-823), consisting domains from scFv fragments of 4D5 and A21, was synthesized at Genescript Inc. and carries additionally BamHI/HindIII cloning sites for directional cloning into pcDNA3 (see below).

(138) The diabody construct A21H_4D5LH_A21L (SEQ ID 79) consists of a first moiety consisting of the A21 heavy chain connected to the 4D5 light chain by a glycine/serine linker characterized by SEQ ID 51, and second moiety consisting of the 4D5 heavy chain connected to the A21 light chain by a glycine/serine linker characterized by SEQ ID 51, wherein the first moiety is connected to the second moiety by a glycine/serine linker characterized by SEQ ID 54 (FIG. 19A).

(139) Expression of Diabody Constructs in CHO Cells

(140) For the expression of the diabody construct A21H_4D5LH_A21L a vector plasmid based on pcDNA3.1(+) Hygro has been constructed. A poly linker (multiple cloning site) was synthesized that carries a N-terminal signal sequence of the mouse Ig Kappa light chain followed by BamHI/HindIII cloning site and a C-terminal 6His-tag (FIG. 19B). The vector was termed pcDNA3.1 Seq mIg.

(141) TABLE-US-00010 METDTLLLWVLLLWVPGSTGS(SEQID97)- embedded image(SEQID98) METDTLLLWVLLLWVPGST:mouseIgKappalightchain signalsequence(SEQID115) GS:BamHIsite KL:HindIIIsite HHHHHH:6xHisTag(SEQID114)

(142) Chinese hamster ovarian cells (CHO) FreeStyle from Invitrogen adapted for serum free suspension growth have been used for transient expression of the diabody construct. The diabody plasmid (pcDNA3.1 Seq mIg A21H_4D5LH_A21L) was transfected into CHO cells by TransIT-PRO (Mirus) transfection reagent using the manufacturer's protocol. Expression was performed in bioreactors (Sigma) for 1 week in CHO-FreeStyle medium (Invitrogen).

(143) Purification of Diabody Constructs from Supernatant of CHO Cells

(144) After expression, the supernatant was collected by centrifugation, filtered and concentrated to a small volume. The supernatant was dialyzed against Tris buffer (50 mM Tris base, 150 mM NaCl, pH 7.5) and afterwards adjusted to 20 mM imidazole, 400 mM NaCl, 10% glycerol and loaded on a Ni-NTA bench top column. The column was washed with 30 CV of Tris buffer containing 20 mM imidazole, 400 mM NaCl, 10% glycerol, pH 7.5, 30 CV of Tris buffer and eluted in 2 CV Tris buffer pH 7.5 containing 300 mM imidazole. Samples were concentrated and dialyzed against HEPES buffer (25 mM HEPES, 150 mM NaCl, pH 7.5).

(145) Anti-Tumor Activity of the Bispecific HER2 Binding Agents in Comparison to Trastuzumab

(146) To test the cytotoxic activity of the bispecific HER2 binding agents described above, XTT-viability assay were performed as described in example 1. FIG. 20 show the results of the tests in BT474 cells, and FIG. 21 the results of the tests in HCC1419 cells, wherein CTRL means control, no addition; A21 the scFv fragment A21, 4D5 the scFv fragment 4D5; A21+4D5, a mixture of scFv fragment A21 and scFv fragment 4D5; and TZB trastuzumab. Note that the diabody (SEQ ID 79) was used at only 10 nM in the experiments shown in FIG. 21, while all other agents were used at 100 nM.

(147) These results show that the principle of connecting a binder of domain 1 of HER to a binder of domain of HER2 by a linker leads in order to obtain a compound with strong cytotoxic and/or anti-proliferative effects does work, no matter whether the binder is or comprises an antibody fragment or a DARPin.

(148) Additionally TUNEL assays as described in example 1 were performed with the above mentioned bispecific HER2 binding agents. As shown in FIG. 22, the percentage of TUNEL-positive cells is significantly higher for the tested bispecific agents than for trastuzumab. These results were verified by Western blot analysis, wherein the apoptosis was detected by the cleavage of Poly ADP Ribose Polymerase (FIG. 23). The Western blot analysis was performed as described in example 1.

(149) In summary, it could be shown that the bispecific HER2 binding agents comprising one or two antibody fragments are able to trigger apoptosis of the targeted cell much better than trastuzumab.

Example 6: Bispecific HER2 Bindings Agent Comprising Two DARPins Connected by a Shared Helix

(150) The principle of the bispecific constructs, namely that an HER2_I and an HER2_IV binder are fused in order to bring the respective domains of two different HER2-molecules into proximity, does in principle work with flexible linkers of different lengths. As an alternative to this, DARPin constructs have been created in which the two DARPins have been fused rigidly in different angles and tested in cell viability assay as described in example 1.

(151) All 9 tested constructs 9.29_SH_G3 #2 (SEQ ID 102), 9.29_SH_G3 #6 (SEQ ID 103), 9.29_SH_G3 #9 (SEQ ID 104), 9.29_SH_G3 #10 (SEQ ID 105), 9.29_G3 #11 (SEQ ID 106), 9.29_SH_G3 #12 (SEQ ID 107), 9.29_SH_G3 #13 (SEQ ID 108), 9.29_SH_G3 #14 (SEQ ID 109), and 9.29_SH_G3 #15 (SEQ ID 110) have strong anti-proliferative activity in cell viability assays with HER2-dependent cancer cells (BT474), however to varying degrees.

(152) Without wishing to be bound by theory, it is supposed that the target (HER2) can orient in various orientations over the membrane insertion point. Still, in all different orientations, the two transmembrane helices of the bound receptors will be kept at a distance sufficient to inactivate the kinase activity. This blueprint allows some flexibility in the epitopes bound on HER2_I and HER2_IV and in the orientation with which these epitopes are bound.