Bcl-2 Associated Athanogene 3/BAG3 [MD337]

BAG3 (Bcl-2-associated athanogene 3), also known as BIS or CAIR-1, is a 575 amino acid protein that contains one C-terminal Bag domain and two N-terminal WW domains. Like other members of the Bag family, BAG3 functions to inhibit the chaperone activity of HSP 70, specifically by promoting the release of HSP 70-bound substrates. Additionally, BAG3 exhibits anti-apoptotic activity via cell cycle control, suggesting a possible role for BAG3 in tumor progression. The gene encoding BAG3 maps to human chromosome 10, which houses over 1,200 genes and comprises nearly 4.5% of the human genome. Inhibits the chaperone activity of HSP70/HSC70 by promoting substrate release. Defects in BAG3 are the cause of myopathy myofibrillar BAG3-related (MFM-BAG3). A neuromuscular disorder that results in early-onset, severe, progressive, diffuse muscle weakness associated with cardiomyopathy, severe respiratory insufficiency during adolescence, and a rigid spine in some patients.