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Protein Page:
BAG3 (human)

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. Note: This description may include information from UniProtKB.
Protein type: Apoptosis
Chromosomal Location of Human Ortholog: 10q26.11
Cellular Component: cell-cell adherens junction; cytoplasm; cytosol; plasma membrane
Molecular Function: adenyl-nucleotide exchange factor activity; protein binding
Biological Process: induction of apoptosis via death domain receptors
Disease: Cardiomyopathy, Dilated, 1hh; Myopathy, Myofibrillar, 6
Reference #:  O95817 (UniProtKB)
Alt. Names/Synonyms: BAG family molecular chaperone regulator 3; BAG-3; BAG-family molecular chaperone regulator-3; BAG3; Bcl-2-associated athanogene 3; Bcl-2-binding protein Bis; BCL2-associated athanogene 3; BCL2-binding athanogene 3; BIS; CAIR-1; Docking protein CAIR-1; MGC104307
Gene Symbols: BAG3
Molecular weight: 61,595 Da
Basal Isoelectric point: 6.46  Predict pI for various phosphorylation states
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Protein Structure Not Found.
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