Described are compounds for targeting proteases, e.g. serine proteases and their use in the diagnostic methods and methods for treatment of respiratory diseases such as cystic fibrosis. The compounds have the structure [H][B]-[A]; wherein [H] is a hydrophilic group, [B] is a subsite recognition group and [A] is a binding group; wherein A has the formula: C(0)CH.sub.2NR.sup.1COOR.sup.2 and wherein [B] has the structure: (i) [COCH.sub.2NR.sup.3]m-, or (ii) -[AA1-AA2]- or (iii) -(AA1-C0-CH.sub.2NR.sup.3) or (iv) (COCH.sub.2NR.sup.3-AA1)- or (v) (C0CH.sub.2NR.sup.4-AA1-AA3)-.

A method to determine the severity of a disease of chronic inflammation in a patient, comprising the steps of (1) collection or preparation of a bodily fluid, tissue or lavage and (2) measurement of ALX receptor ligands or ALX receptor expression in the fluid, tissue, or lavage, wherein the level of ALX receptor ligands predicts a clinical outcome or choice of treatment modality, is disclosed.

The invention relates to an assay for diagnosing a disease or affliction that affects fluid uptake or secretion or for studying the effectiveness of one or more drugs for treating the disease or affliction, wherein the assay comprises measuring swelling of one or more organoids.

The present invention provides novel mutations identified in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that can be used for a more accurate diagnosis of cystic fibrosis (CF) and CF related disorders. Methods for testing a sample obtained from a subject to determine the presence of one or more mutations in the CFTR gene are provided wherein the presence of one or more mutations indicates that the subject has CF or a CF related disorder, or is a carrier of a CFTR mutation.

The present invention is related to novel methods for diagnosing and treating acute pulmonary exacerbation in subjects in need thereof.

The invention relates to an assay for diagnosing a disease or affliction that affects fluid uptake or secretion or for studying the effectiveness of one or more drugs for treating the disease or affliction, wherein the assay comprises measuring swelling of one or more organoids.

The present invention is directed to modified CFTR proteins or fragments thereof that contain single or multiple amino acid mutations to improve the structural stability of such CFTR proteins and/or fragments. Specifically, the modified CFTR proteins or fragment thereof differ from the wild-type human CFTR protein or fragment thereof by the presence of four or more mutations selected from V150D, M470V, S492P, F494N, 5495P, A534P, I539T, G550E, G551D, R553Q, R555K, Q637R, 51255L, K1334G, 51359A, E1371Q, H14025, Q1411D, and any combination thereof, such that the stability of the polypeptide is increased relative to that of the wild-type human CFTR polypeptide or fragment thereof.

Systems, methods, devices, and kits are described that can be used to distinguish cystic fibrosis patients from healthy individuals and from lung cancer patients. The systems, methods, devices, and kits utilize one or more serum biomarkers.

A method of determining if a patient having cystic fibrosis has an increased risk of having or developing pulmonary exacerbations is described. The method includes determining the level of one or more metabolites associated with pulmonary exacerbations in a sweat sample from the patient, and characterizing the patient as having an increased risk of having or developing pulmonary exacerbations if one or more metabolites associated with pulmonary exacerbation are significantly different from a control value. A method of evaluating the response of a patient having pulmonary exacerbations to treatment is also described.

The present disclosure is directed to methods of identifying CFTR modulators using non-mutant or mutant CFTR expressing cells in the presence of a CFTR amplifier compound.