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|Applications||ELISA IHC WB|
|Antigen/Gene or Protein Targets||BAG family molecular chaperone regulator 3|
|Synonyms||BAG3, Bcl-2-associated athanogene 3, Bcl-2-binding protein Bis, Docking protein CAIR-1|
Anti-BCL2-Associated Athanogene 3 (BAG3) [V65P1E8*D2] is specific for human BAG3. BAG3 is involved in chaperone-assisted selective autophagy. Inhibits the chaperone activity of HSP70/HSC70 by promoting substrate release. Has anti-apoptotic activity.
In muscle cells, BAG3 cooperates with the molecular chaperones Hsc70 and HspB8 to induce the degradation of mechanically damaged cytoskeleton components in lysosomes. This process is called chaperone-assisted selective autophagy (CASA) and is essential for maintaining muscle activity.
Defects in BAG3 are the cause of myopathy myofibrillar BAG3-related (MFM-BAG3) [MIM:612954]. 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.
|Immunogen||Ovalbumin-conjugated synthetic peptide MTDTPGNPAAP|
|Research Area||Apoptosis and Programmed Cell Death, Cancer, Cardiovascular, Metabolism|
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