The present disclosure relates to an inhibitor of Dynamin 2 for use in the treatment of centronuclear myopathies. The present disclosure relates to pharmaceutical compositions containing Dynamin 2 inhibitor and to their use for the treatment of centronuclear myopathies. It also deals with a method for identifying or screening molecules useful in the treatment of a centronuclear myopathy.

Disclosed herein are devices including a substrate, one or more regions of electrically active material, and optionally at least one electrode. Also disclosed herein are devices or systems including a substrate, one or more regions of electrically active material including muscle cells and neuronal cells, and optionally at least one electrode. In some embodiments, the muscle cells and neuronal cells form one or more neuromuscular junctions at defined locations on the electrically active material. Also disclosed are methods of using the devices and systems for analyzing the effect of compounds on neuronal cell, muscle cell and/or neuromuscular junction activity.

Provided are a composition for diagnosing vascular disease including an agent measuring a level of interleukin 12 receptor β2 protein in the blood, and a kit for diagnosing vascular disease including the same. Further, provided is a method for diagnosing vascular disease, the method including the step of measuring a level of interleukin 12 receptor β2 protein in a blood sample separated from an individual suspected of having vascular disease. Furthermore, provided are a composition for preventing or treating vascular disease including an interleukin 12 receptor β2 activity inhibitor, and a method of screening a therapeutic agent for vascular disease, the method including the step of treating smooth muscle cells with a test agent for vascular disease treatment and measuring an expression level of interleukin 12 receptor β2.

Provided herein are Si-based materials, methods of making the Si-based materials, and methods for using the Si-based materials. In embodiments, a silicon-based material comprises an aggregate of particles, the particles comprising an ordered array of nanostructures, the nanostructures comprising amorphous silicon, wherein at least some pairs of adjacent nanostructures are connected by one or more bridges comprising amorphous silicon, the one or more bridges extending from the surface of one nanostructure of the pair to the surface of the other nanostructure in the pair.

Herein is reported a method for determining non-specific clearance of an antibody comprising the steps of incubating the antibody, which is conjugated to a pH-sensitive fluorescent dye, with primary human endothelial cells, and determining the fluorescence intensity of the primary human endothelial cells, whereby an increase of the fluorescence intensity of the primary human endothelial cells above background level is indicative for non-specific clearance of the antibody.

Measurements in test tissue and drug-treated tissue can be used to determine an effect of the drug, e.g., on cardiomyocytes. Optical measurements of test tissue (e.g., including immature or animal CMs) can be complemented with observations of the extracellular potential, e.g., using multi electrode arrays (MEAs), as part of accurately estimating the current density of an ion channel, e.g., the sodium channels. The estimating of an ion current density (e.g., by inversion) of the ion (e.g., sodium) current can also use an observation of the conduction velocity, which can be computed using measurements of extracellular waves across electrodes. Example optical measurements can be of a transmembrane voltage (Vm) and intracellular calcium concentration (Cai). An example electrical measurement can be of an extracellular potential (U).

The present subject matter provides a microfluidic device that enables the precise and repeatable three dimensional and compartmentalized coculture of muscle cells and neuronal cells. Related apparatus, systems, techniques, and articles are also described.

The present invention relates to a method for the generation of functional skeletal muscle fibers innervated by motoneurons, from pluripotent stem cells.

The present invention is in the field of an all-in-one microchamber for 3D muscular tissues, wherein at least one 3D microenvironment is present, a method of producing said device using silicon-based technology, and a use of said device in various applications, typically a biological cell experiment, such as a cell or organ-on-a-chip experiment, and lab-on-a-chip experiment, and use of the device as a micro-reactor.

The present invention relates to generating hiPSC-derived cardiomyocytes and embryoid bodies that recapitulate the disease phenotype of Long QT Syndrome and their use in developing pharmacological treatments thereof. The present invention also includes the use of a compound which inhibits the ubiquitin-proteasome pathway for the preparation of a medicament for the prophylaxis or treatment of a disease associated with prolonged ventricular repolarization (cardiac arrhythmia) caused by one or more mutations in the amino acid sequence of the hERG potassium channel.