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BAG family molecular chaperone regulator 1; BAG-1; Bcl-2-associated athanogene 1; glucocorticoid receptor-associated protein RAP46 (BAG1, HAP)

Function: - inhibits the chaperone activity of HSP70/HSC70 by promoting substrate release - inhibits the pro-apoptotic function of PPP1R15A, & has anti-apoptotic activity - bcl-2 binding protein - markedly increases the anti-cell death function of BCL2 induced by various stimuli - ubiquitinated; mediated by SIAH1 or SIAH2 & leading to its subsequent proteasomal degradation (probable) - binds to the ATPase domain of HSP70/HSC chaperones - binds to BCL2 & NR3C1 - interacts with N-terminal region of STK19 - interacts with PPP1R15A - isoform 2 doesn't interact with HSP70/HSC or BCL2 Structure: - contains 1 BAG domain - contains 1 ubiquitin-like domain Compartment: - isoform 2: cytoplasm, nucleus; localizes to the cytoplasm & shuttles into the nucleus in response to heat shock Alternative splicing: named isoforms=2 Pathology: - defects/overactivity in BAG1 may be linked to the cryptophthalmos syndrome (Fraser syndrome)

Interactions

molecular events

Related

bag1 gene

General

anti apoptotic protein bcl-2 binding athanogene (bag) protein

Properties

SIZE: entity length = 345 aa MW = 39 kD COMPARTMENT: cytoplasm cell nucleus MOTIF: arginine-rich region {4-82} MOTIF: arginine residue (SEVERAL) 7 X 6 AA tandem repeat of E-E-X(4) {96-137} MOTIF: consensus repeat {96-101} consensus repeat {102-107} consensus repeat {108-113} consensus repeat {114-119} consensus repeat {120-125} consensus repeat {126-131} consensus repeat {132-137} Ubiquitin-like {144-224} PPP1R15A interaction {216-345} MOTIF: BAG {246-326}

Database Correlations

OMIM 601497 UniProt Q99933 PFAM correlations Entrez Gene 573

References

  1. UniProt :accession Q99933
  2. Atlas of Genetics & Cytogenetics in Oncology & Haematology http://atlasgeneticsoncology.org//genes/BAG1ID742ch9p13.html
  3. Takayama S, Sato T, Krajewski S, Kochel K, Irie S, Millan JA, Reed JC. Cloning and functional analysis of BAG-1: a novel Bcl-2- binding protein with anti-cell death activity. Cell. 1995 Jan 27;80(2):279-84. PMID: 7834747
  4. Entrez Gene :accession 573

Component-of

molecular complex