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BAG3 BAG cochaperone 3 [ Homo sapiens (human) ]

Gene ID: 9531, updated on 7-Apr-2024

Summary

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Official Symbol
BAG3provided by HGNC
Official Full Name
BAG cochaperone 3provided by HGNC
Primary source
HGNC:HGNC:939
See related
Ensembl:ENSG00000151929 MIM:603883; AllianceGenome:HGNC:939
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Homo sapiens
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
Also known as
BIS; MFM6; BAG-3; CAIR-1
Summary
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]
Expression
Ubiquitous expression in heart (RPKM 32.6), placenta (RPKM 26.6) and 25 other tissues See more
Orthologs
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Genomic context

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Location:
10q26.11
Exon count:
4
Annotation release Status Assembly Chr Location
RS_2023_10 current GRCh38.p14 (GCF_000001405.40) 10 NC_000010.11 (119651380..119677819)
RS_2023_10 current T2T-CHM13v2.0 (GCF_009914755.1) 10 NC_060934.1 (120548963..120575488)
105.20220307 previous assembly GRCh37.p13 (GCF_000001405.25) 10 NC_000010.10 (121410892..121437331)

Chromosome 10 - NC_000010.11Genomic Context describing neighboring genes Neighboring gene RAD1 pseudogene 1 Neighboring gene MPRA-validated peak1112 silencer Neighboring gene ReSE screen-validated silencer GRCh37_chr10:121396416-121396639 Neighboring gene ribosomal protein S8 pseudogene 4 Neighboring gene H3K27ac hESC enhancer GRCh37_chr10:121410030-121410984 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr10:121425241-121425794 Neighboring gene ReSE screen-validated silencer GRCh37_chr10:121427873-121428054 Neighboring gene uncharacterized LOC124902513 Neighboring gene ReSE screen-validated silencer GRCh37_chr10:121443219-121443398 Neighboring gene thioredoxin pseudogene 1

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Expression

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  • Project title: HPA RNA-seq normal tissues
  • Description: RNA-seq was performed of tissue samples from 95 human individuals representing 27 different tissues in order to determine tissue-specificity of all protein-coding genes
  • BioProject: PRJEB4337
  • Publication: PMID 24309898
  • Analysis date: Wed Apr 4 07:08:55 2018

Bibliography

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Related articles in PubMed

  1. BAG3: Nature's Quintessential Multi-Functional Protein Functions as a Ubiquitous Intra-Cellular Glue. Brenner CM, et al. Cells, 2023 Mar 19. PMID 36980278, Free PMC Article
  2. Unraveling the mystery: How bad is BAG3 in hematological malignancies? Liu Q, et al. Biochim Biophys Acta Rev Cancer, 2022 Sep. PMID 35985611
  3. Therapeutic induction of Bcl2-associated athanogene 3-mediated autophagy in idiopathic pulmonary fibrosis. Chillappagari S, et al. Clin Transl Med, 2022 Jul. PMID 35834635, Free PMC Article
  4. Lutein induces an inhibitory effect on the malignant progression of pancreatic adenocarcinoma by targeting BAG3/cholesterol homeostasis. Han L, et al. J Biochem Mol Toxicol, 2022 Feb. PMID 34783111
  5. BAG3 induces α-SMA expression in human fibroblasts and its over-expression correlates with poorer survival in fibrotic cancer patients. De Marco M, et al. J Cell Biochem, 2022 Jan. PMID 34741483, Free PMC Article

See all (318) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. BAG3 regulates the specificity of the recognition of specific MAPT species by NBR1 and SQSTM1.
    Title: BAG3 regulates the specificity of the recognition of specific MAPT species by NBR1 and SQSTM1.
  2. Distal hereditary motor neuronopathy as a new phenotype associated with variants in BAG3.
    Title: Distal hereditary motor neuronopathy as a new phenotype associated with variants in BAG3.
  3. Dysregulated Autophagy and Sarcomere Dysfunction in Patients With Heart Failure With Co-Occurrence of P63A and P380S BAG3 Variants.
    Title: Dysregulated Autophagy and Sarcomere Dysfunction in Patients With Heart Failure With Co-Occurrence of P63A and P380S BAG3 Variants.
  4. BAG3: Nature's Quintessential Multi-Functional Protein Functions as a Ubiquitous Intra-Cellular Glue.
    Title: BAG3: Nature's Quintessential Multi-Functional Protein Functions as a Ubiquitous Intra-Cellular Glue.
  5. MiR-135a-5p suppresses breast cancer cell proliferation, migration, and invasion by regulating BAG3.
    Title: MiR-135a-5p suppresses breast cancer cell proliferation, migration, and invasion by regulating BAG3.
  6. Unraveling the mystery: How bad is BAG3 in hematological malignancies?
    Title: Unraveling the mystery: How bad is BAG3 in hematological malignancies?
  7. Therapeutic induction of Bcl2-associated athanogene 3-mediated autophagy in idiopathic pulmonary fibrosis.
    Title: Therapeutic induction of Bcl2-associated athanogene 3-mediated autophagy in idiopathic pulmonary fibrosis.
  8. BAG3 protects chondrocytes against lumbar facet joint osteoarthritis by regulating autophagy and apoptosis.
    Title: BAG3 protects chondrocytes against lumbar facet joint osteoarthritis by regulating autophagy and apoptosis.
  9. BAG3 Regulation of RAB35 Mediates the Endosomal Sorting Complexes Required for Transport/Endolysosome Pathway and Tau Clearance.
    Title: BAG3 Regulation of RAB35 Mediates the Endosomal Sorting Complexes Required for Transport/Endolysosome Pathway and Tau Clearance.
  10. Metformin rescues muscle function in BAG3 myofibrillar myopathy models.
    Title: Metformin rescues muscle function in BAG3 myofibrillar myopathy models.
Submit: New GeneRIF Correction See all GeneRIFs (197)

Phenotypes

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Find tests for this gene in the NIH Genetic Testing Registry (GTR)

Review eQTL and phenotype association data in this region using PheGenI

Copy number response

Description
Copy number response
Triplosensitivity

No evidence available (Last evaluated 2015-02-12)

ClinGen Genome Curation Page
Haploinsufficency

Sufficient evidence for dosage pathogenicity (Last evaluated 2015-02-12)

ClinGen Genome Curation PagePubMed

EBI GWAS Catalog

Description
A genome-wide association study identifies two loci associated with heart failure due to dilated cardiomyopathy.
EBI GWAS Catalog

Variation

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See variants in ClinVar

See studies and variants in dbVar

See Variation Viewer (GRCh37.p13)

See Variation Viewer (GRCh38)

Genotypes

HIV-1 interactions

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Protein interactions

Protein Gene Interaction Pubs
Tat tat HIV-1 Tat upregulates the levels of BAG3 in human glioblastoma cells, and BAG3 silencing results in highly reducing Tat-induced upregulation of LC3-II and increasing the appearance of sub G0/G1 apoptotic cells PubMed

Go to the HIV-1, Human Interaction Database

Interactions

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Products Interactant Other Gene Complex Source Pubs Description

General gene information

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Markers

Homology

Clone Names

  • MGC104307

Gene Ontology Provided by GOA

Function Evidence Code Pubs
enables adenyl-nucleotide exchange factor activity IBA
Inferred from Biological aspect of Ancestor
more info
  
enables adenyl-nucleotide exchange factor activity IDA
Inferred from Direct Assay
more info
 PubMed 
enables cadherin binding HDA PubMed 
enables dynein intermediate chain binding NAS
Non-traceable Author Statement
more info
 PubMed 
enables protein binding IPI
Inferred from Physical Interaction
more info
 PubMed 
enables protein carrier chaperone TAS
Traceable Author Statement
more info
 PubMed 
enables protein-containing complex binding IEA
Inferred from Electronic Annotation
more info
  
enables protein-folding chaperone binding IBA
Inferred from Biological aspect of Ancestor
more info
  
enables protein-folding chaperone binding IPI
Inferred from Physical Interaction
more info
 PubMed 
Process Evidence Code Pubs
involved_in aggresome assembly TAS
Traceable Author Statement
more info
 PubMed 
involved_in autophagosome assembly IEA
Inferred from Electronic Annotation
more info
  
involved_in cellular response to heat IDA
Inferred from Direct Assay
more info
 PubMed 
involved_in cellular response to mechanical stimulus IEA
Inferred from Electronic Annotation
more info
  
involved_in cellular response to unfolded protein IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in chaperone-mediated autophagy IEA
Inferred from Electronic Annotation
more info
  
involved_in extrinsic apoptotic signaling pathway in absence of ligand IEA
Inferred from Electronic Annotation
more info
  
acts_upstream_of_or_within extrinsic apoptotic signaling pathway via death domain receptors IDA
Inferred from Direct Assay
more info
 PubMed 
involved_in muscle cell cellular homeostasis IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in muscle cell cellular homeostasis IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in negative regulation of apoptotic process NAS
Non-traceable Author Statement
more info
 PubMed 
involved_in negative regulation of striated muscle cell apoptotic process IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in negative regulation of striated muscle cell apoptotic process IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in positive regulation of aggrephagy IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in positive regulation of protein export from nucleus IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in positive regulation of protein import into nucleus IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in protein folding NAS
Non-traceable Author Statement
more info
 PubMed 
involved_in protein stabilization IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in protein stabilization IDA
Inferred from Direct Assay
more info
 PubMed 
acts_upstream_of protein transport along microtubule TAS
Traceable Author Statement
more info
 PubMed 
involved_in striated muscle cell apoptotic process IEA
Inferred from Electronic Annotation
more info
  
Component Evidence Code Pubs
located_in Z disc IEA
Inferred from Electronic Annotation
more info
  
located_in aggresome TAS
Traceable Author Statement
more info
 PubMed 
is_active_in cytoplasm IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in cytoplasm IDA
Inferred from Direct Assay
more info
 PubMed 
is_active_in cytosol IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in cytosol IDA
Inferred from Direct Assay
more info
 PubMed 
located_in cytosol TAS
Traceable Author Statement
more info
  
is_active_in membrane IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in nucleoplasm IDA
Inferred from Direct Assay
more info
  
is_active_in nucleus IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in nucleus IDA
Inferred from Direct Assay
more info
 PubMed 
part_of protein folding chaperone complex IDA
Inferred from Direct Assay
more info
 PubMed 
located_in stress fiber IEA
Inferred from Electronic Annotation
more info
  

General protein information

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Preferred Names
BAG family molecular chaperone regulator 3
Names
BCL2 associated athanogene 3
BCL2-binding athanogene 3
Bcl-2-binding protein BIS transcript 2
bcl-2-binding protein Bis
docking protein CAIR-1

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

Genomic

  1. NG_016125.1 RefSeqGene

    Range
    5001..31450
    Download
    GenBank, FASTA, Sequence Viewer (Graphics), LRG_742

mRNA and Protein(s)

  1. NM_004281.4 → NP_004272.2  BAG family molecular chaperone regulator 3

    See identical proteins and their annotated locations for NP_004272.2

    Status: REVIEWED

    Source sequence(s)
    AF071218, BC006418
    Consensus CDS
    CCDS7615.1
    UniProtKB/Swiss-Prot
    A8K5L8, O95817, Q3B763, Q9NT20, Q9P120
    UniProtKB/TrEMBL
    Q53GY1
    Related
    ENSP00000358081.4, ENST00000369085.8
    Conserved Domains (2) summary
    smart00456
    Location:21 → 53
    WW; Domain with 2 conserved Trp (W) residues
    smart00264
    Location:421 → 498
    BAG; BAG domains, present in regulator of Hsp70 proteins

RefSeqs of Annotated Genomes: GCF_000001405.40-RS_2023_10

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCh38.p14 Primary Assembly

Genomic

  1. NC_000010.11 Reference GRCh38.p14 Primary Assembly

    Range
    119651380..119677819
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_005270287.2 → XP_005270344.1  BAG family molecular chaperone regulator 3 isoform X1

    UniProtKB/TrEMBL
    Q53GY1
    Conserved Domains (2) summary
    smart00456
    Location:21 → 53
    WW; Domain with 2 conserved Trp (W) residues
    smart00264
    Location:420 → 497
    BAG; BAG domains, present in regulator of Hsp70 proteins

Alternate T2T-CHM13v2.0

Genomic

  1. NC_060934.1 Alternate T2T-CHM13v2.0

    Range
    120548963..120575488
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_054367219.1 → XP_054223194.1  BAG family molecular chaperone regulator 3 isoform X1

Nucleotide Protein
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
O95817.3 GenPept UniProtKB/Swiss-Prot:O95817

Gene LinkOut

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