Article
BAG3 directly interacts with mutated alphaB-crystallin to suppress its aggregation and toxicity.
PloS one - 15 Mar 2011
Hishiya Akinori, Salman Mortada Najem, Carra Serena, Kampinga Harm H, Takayama Shinichi
Abstract excerpt
A homozygous disruption or genetic mutation of the bag3 gene causes progressive myofibrillar myopathy in mouse and human skeletal and cardiac muscle disorder while mutations in the small heat shock protein αB-crystallin gene (CRYAB) are reported to be responsible for myofibrillar myopathy. Here, we demonstrate that BAG3 directly binds to wild-type αB-crystallin and the αB-crystallin mutant R120G, via the...
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