BAG3

Autosomal dominant inheritance

Bag3 is usually an early onset muscular dystrophy.

Myofibrillar myopathy-6 is an autosomal dominant severe neuromuscular disorder characterized by onset in the first decade of rapidly progressive generalized and proximal muscle weakness, respiratory insufficiency, cardiomyopathy, and skeletal deformities related to muscle weakness. Muscle biopsy shows fiber-type grouping, disruption of the Z lines, and filamentous inclusions, and sural nerve biopsy shows a neuropathy, often with giant axonal neurons. Most patients are severely affected by the second decade and need cardiac transplant, ventilation, and/or a wheelchair.

NIH Link This link is for the National Library of Medicine.

Alexander Way This link is a leading non profit searching for a cure for Bag3 MFM.

Bag3 Research Foundation in Germany