A new collaboration agreement has been signed between the Swedish company NeuroVive Pharmaceutical AB and Karolinska Institutet, Stockholm in order to develop NeuroVive’s cyclophilin inhibitor compound NV556 for the treatment of mitochondrial myopathy, an area of high unmet medical need of new and effective treatment options.

NeuroVive Pharmaceutical AB and Karolinska Institutet will collaborate to develop the cyclophilin inhibitor NV556 for the treatment of mitochondrial myopathy

The collaboration will allow NeuroVive to reinforce its presence in the genetic mitochondrial disease key focus area where the company is also developing NVP015 compounds in mitochondrial respiratory chain diseases. The NV556 compound has a different and complementary mode of action than NVP015. The new compound is expected to have higher specificity and tolerability than ciclosporin, which may facilitate better optimization of dosage. Ciclosporin is another cyclophilin inhibitor those mitochondrial protective effects mediated through inhibition of cyclophilin D have already been demonstrated by the research team led by Prof. Håkan Westerblad at Karolinska Institutet.

Mitochondrial myopathies are a group of neuromuscular diseases caused by damage to the mitochondria, the small energy factories found inside almost all the cells in the body. The symptoms of mitochondrial myopathies include muscle weakness, exercise intolerance and fatigue, and are often accompanied by other symptoms of genetic mitochondrial disorders such as heart failure or rhythm disturbances, dementia, movement disorders, stroke-like episodes, deafness, blindness, droopy eyelids, limited mobility of the eyes, vomiting, and seizures. The prognosis for these disorders ranges in severity from progressive weakness to death.