BCL2-Associated Athanogene 3 (BAG3) Human Recombinant Protein

BAG proteins compete with Hip for binding to the Hsc70/Hsp70 ATPase domain and promote substrate release. All the BAG proteins have an approximately 45-amino acid BAG domain near the C terminus but differ markedly in their N-terminal regions. The protein encoded by this gene contains a WW domain in the N-terminal region and a BAG domain in the C-terminal region. The BAG domains of BAG1, BAG2, and BAG3 interact specifically with the Hsc70 ATPase domain in vitro and in mammalian cells. All 3 proteins bind with high affinity to the ATPase domain of Hsc70 and inhibit its chaperone activity in a Hip-repressible manner. [provided by RefSeq, Jul 2008]

BAG3: Inhibits the chaperone activity of HSP70/HSC70 by promoting substrate release. Has anti-apoptotic activity. Defects in BAG3 are the cause of myopathy myofibrillar type 6 (MFM6). 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. At ultrastructural level, muscle fibers display structural alterations consisting of replacement of the normal myofibrillar markings by small, dense granules, or larger hyaline masses, or amorphous material. Defects in BAG3 are the cause of cardiomyopathy dilated type 1HH (CMD1HH). CMD1HH is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.

Protein type: Apoptosis

Chromosomal Location of Human Ortholog: 10q25.2-q26.2

Cellular Component: neuron projection; cytoplasm; plasma membrane; cytosol; Z disc

Molecular Function: protein binding; chaperone binding; protein complex binding

Biological Process: induction of apoptosis via death domain receptors; protein stabilization; protein folding; spinal cord development; brain development; negative regulation of apoptosis

Disease: Myopathy, Myofibrillar, 6; Cardiomyopathy, Dilated, 1hh