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Protein

BAG family molecular chaperone regulator 3

Gene

BAG3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Inhibits the chaperone activity of HSP70/HSC70 by promoting substrate release. Has anti-apoptotic activity.

GO - Molecular functioni

  • adenyl-nucleotide exchange factor activity Source: MGI
  • cadherin binding involved in cell-cell adhesion Source: BHF-UCL

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Chaperone

Keywords - Biological processi

Apoptosis

Enzyme and pathway databases

BioCyciZFISH:ENSG00000151929-MONOMER.
ReactomeiR-HSA-3371453. Regulation of HSF1-mediated heat shock response.
SignaLinkiO95817.
SIGNORiO95817.

Names & Taxonomyi

Protein namesi
Recommended name:
BAG family molecular chaperone regulator 3
Short name:
BAG-3
Alternative name(s):
Bcl-2-associated athanogene 3
Bcl-2-binding protein Bis
Docking protein CAIR-1
Gene namesi
Name:BAG3
Synonyms:BIS
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 10

Organism-specific databases

HGNCiHGNC:939. BAG3.

Subcellular locationi

GO - Cellular componenti

  • cell-cell adherens junction Source: BHF-UCL
  • cytoplasm Source: HPA
  • cytosol Source: MGI
  • neuron projection Source: Ensembl
  • plasma membrane Source: HPA
  • Z disc Source: Ensembl
Complete GO annotation...

Pathology & Biotechi

Involvement in diseasei

Myopathy, myofibrillar, 6 (MFM6)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA form of myofibrillar myopathy, a group of chronic neuromuscular disorders characterized at ultrastructural level by disintegration of the sarcomeric Z disc and myofibrils, and replacement of the normal myofibrillar markings by small dense granules, or larger hyaline masses, or amorphous material. MFM6 is characterized by early-onset of severe, progressive, diffuse muscle weakness associated with cardiomyopathy, severe respiratory insufficiency during adolescence, and a rigid spine in some patients.
See also OMIM:612954
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_063089209P → L in MFM6; interferes with the differentiation of skeletal muscle cells; does not cause functional alterations in cardiomyocyte cells. 3 PublicationsCorresponds to variant rs121918312dbSNPEnsembl.1
Cardiomyopathy, dilated 1HH (CMD1HH)3 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
See also OMIM:613881
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_06547971R → W in CMD1HH. 1 PublicationCorresponds to variant rs387906874dbSNPEnsembl.1
Natural variantiVAR_066781218R → W in CMD1HH; interferes with the assembly of Z-disks; increases stress-induced apoptosis. 1 PublicationCorresponds to variant rs397514506dbSNPEnsembl.1
Natural variantiVAR_066785455E → K in CMD1HH. 1 PublicationCorresponds to variant rs397516881dbSNPEnsembl.1
Natural variantiVAR_066786462L → P in CMD1HH; interferes with the assembly of Z-disks; increases stress-induced apoptosis. 1 PublicationCorresponds to variant rs397514507dbSNPEnsembl.1
Natural variantiVAR_066787468V → M in CMD1HH. 1 Publication1
Natural variantiVAR_065480477R → H in CMD1HH. 1 PublicationCorresponds to variant rs387906876dbSNPEnsembl.1

Keywords - Diseasei

Cardiomyopathy, Disease mutation, Myofibrillar myopathy

Organism-specific databases

DisGeNETi9531.
MalaCardsiBAG3.
MIMi612954. phenotype.
613881. phenotype.
OpenTargetsiENSG00000151929.
Orphaneti154. Familial isolated dilated cardiomyopathy.
199340. Muscular dystrophy, Selcen type.
PharmGKBiPA25239.

Polymorphism and mutation databases

BioMutaiBAG3.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources
ChainiPRO_00000888682 – 575BAG family molecular chaperone regulator 3Add BLAST574

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylserineCombined sources1
Modified residuei136PhosphoserineBy similarity1
Modified residuei139Omega-N-methylarginineBy similarity1
Modified residuei173PhosphoserineCombined sources1
Modified residuei198PhosphoserineCombined sources1
Modified residuei261Omega-N-methylarginineBy similarity1
Modified residuei269PhosphoserineCombined sources1
Modified residuei274PhosphoserineCombined sources1
Modified residuei275PhosphoserineCombined sources1
Modified residuei279PhosphoserineCombined sources1
Modified residuei285PhosphothreonineCombined sources1
Modified residuei289PhosphoserineCombined sources1
Modified residuei291PhosphoserineCombined sources1
Modified residuei377PhosphoserineCombined sources1
Modified residuei385PhosphoserineCombined sources1
Modified residuei386PhosphoserineCombined sources1
Modified residuei406PhosphothreonineCombined sources1
Cross-linki445Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO1)Combined sources

Keywords - PTMi

Acetylation, Isopeptide bond, Methylation, Phosphoprotein, Ubl conjugation

Proteomic databases

EPDiO95817.
MaxQBiO95817.
PaxDbiO95817.
PeptideAtlasiO95817.
PRIDEiO95817.

PTM databases

iPTMnetiO95817.
PhosphoSitePlusiO95817.

Miscellaneous databases

PMAP-CutDBO95817.

Expressioni

Gene expression databases

BgeeiENSG00000151929.
CleanExiHS_BAG3.
ExpressionAtlasiO95817. baseline and differential.
GenevisibleiO95817. HS.

Organism-specific databases

HPAiHPA018493.
HPA020586.

Interactioni

Subunit structurei

Binds to the ATPase domain of HSP/HSC70 chaperones. Binds to Bcl-2 and PLC-gamma. Interacts with DNAJB6.1 Publication

Binary interactionsi

WithEntry#Exp.IntActNotes
Q96BE02EBI-747185,EBI-9356686
ARRDC3Q96B675EBI-747185,EBI-2875665
DAZAP2Q150386EBI-747185,EBI-724310
HSPA8Q96IS63EBI-747185,EBI-10289199
HSPB1P047924EBI-747185,EBI-352682
HSPB8Q9UJY18EBI-747185,EBI-739074
LITAFQ997325EBI-747185,EBI-725647
LRRK2Q5S0072EBI-747185,EBI-5323863
MAGED1Q9Y5V35EBI-747185,EBI-716006
PDE9AO760833EBI-747185,EBI-742764
SF3B4Q154275EBI-747185,EBI-348469
SQSTM1Q135013EBI-747185,EBI-307104
STUB1Q9UNE73EBI-747185,EBI-357085
TINF2Q9BSI42EBI-747185,EBI-717399

GO - Molecular functioni

  • cadherin binding involved in cell-cell adhesion Source: BHF-UCL

Protein-protein interaction databases

BioGridi114907. 458 interactors.
DIPiDIP-41273N.
IntActiO95817. 100 interactors.
MINTiMINT-208995.
STRINGi9606.ENSP00000358081.

Structurei

3D structure databases

ProteinModelPortaliO95817.
SMRiO95817.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini20 – 54WW 1PROSITE-ProRule annotationAdd BLAST35
Domaini124 – 154WW 2PROSITE-ProRule annotationAdd BLAST31
Domaini421 – 498BAGPROSITE-ProRule annotationAdd BLAST78

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi180 – 187Poly-Ser8

Sequence similaritiesi

Contains 1 BAG domain.PROSITE-ProRule annotation
Contains 2 WW domains.PROSITE-ProRule annotation

Keywords - Domaini

Repeat

Phylogenomic databases

eggNOGiKOG0940. Eukaryota.
KOG4361. Eukaryota.
COG5021. LUCA.
GeneTreeiENSGT00530000063256.
HOGENOMiHOG000050234.
HOVERGENiHBG003419.
InParanoidiO95817.
KOiK09557.
OMAiVYPQLRP.
OrthoDBiEOG091G08LY.
PhylomeDBiO95817.
TreeFamiTF102013.

Family and domain databases

Gene3Di1.20.58.120. 1 hit.
InterProiIPR003103. BAG_domain.
IPR001202. WW_dom.
[Graphical view]
PfamiPF02179. BAG. 1 hit.
PF00397. WW. 1 hit.
[Graphical view]
SMARTiSM00264. BAG. 1 hit.
SM00456. WW. 1 hit.
[Graphical view]
SUPFAMiSSF51045. SSF51045. 1 hit.
SSF63491. SSF63491. 1 hit.
PROSITEiPS51035. BAG. 1 hit.
PS01159. WW_DOMAIN_1. 1 hit.
PS50020. WW_DOMAIN_2. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

O95817-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MSAATHSPMM QVASGNGDRD PLPPGWEIKI DPQTGWPFFV DHNSRTTTWN
60 70 80 90 100
DPRVPSEGPK ETPSSANGPS REGSRLPPAR EGHPVYPQLR PGYIPIPVLH
110 120 130 140 150
EGAENRQVHP FHVYPQPGMQ RFRTEAAAAA PQRSQSPLRG MPETTQPDKQ
160 170 180 190 200
CGQVAAAAAA QPPASHGPER SQSPAASDCS SSSSSASLPS SGRSSLGSHQ
210 220 230 240 250
LPRGYISIPV IHEQNVTRPA AQPSFHQAQK THYPAQQGEY QTHQPVYHKI
260 270 280 290 300
QGDDWEPRPL RAASPFRSSV QGASSREGSP ARSSTPLHSP SPIRVHTVVD
310 320 330 340 350
RPQQPMTHRE TAPVSQPENK PESKPGPVGP ELPPGHIPIQ VIRKEVDSKP
360 370 380 390 400
VSQKPPPPSE KVEVKVPPAP VPCPPPSPGP SAVPSSPKSV ATEERAAPST
410 420 430 440 450
APAEATPPKP GEAEAPPKHP GVLKVEAILE KVQGLEQAVD NFEGKKTDKK
460 470 480 490 500
YLMIEEYLTK ELLALDSVDP EGRADVRQAR RDGVRKVQTI LEKLEQKAID
510 520 530 540 550
VPGQVQVYEL QPSNLEADQP LQAIMEMGAV AADKGKKNAG NAEDPHTETQ
560 570
QPEATAAATS NPSSMTDTPG NPAAP
Length:575
Mass (Da):61,595
Last modified:January 11, 2001 - v3
Checksum:iA6328A44F37A406A
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti227Q → K in AAD16122 (PubMed:9873016).Curated1
Sequence conflicti237Q → R in AAD16122 (PubMed:9873016).Curated1
Sequence conflicti304Missing in CAB70824 (PubMed:17974005).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_04834471R → Q.1 PublicationCorresponds to variant rs35434411dbSNPEnsembl.1
Natural variantiVAR_06547971R → W in CMD1HH. 1 PublicationCorresponds to variant rs387906874dbSNPEnsembl.1
Natural variantiVAR_06677777P → L.1 PublicationCorresponds to variant rs141355480dbSNPEnsembl.1
Natural variantiVAR_06677894I → F.1 PublicationCorresponds to variant rs145393807dbSNPEnsembl.1
Natural variantiVAR_066779115P → S.1 PublicationCorresponds to variant rs774241343dbSNPEnsembl.1
Natural variantiVAR_048345151C → R.2 PublicationsCorresponds to variant rs2234962dbSNPEnsembl.1
Natural variantiVAR_066780155A → T.1 PublicationCorresponds to variant rs61756328dbSNPEnsembl.1
Natural variantiVAR_063089209P → L in MFM6; interferes with the differentiation of skeletal muscle cells; does not cause functional alterations in cardiomyocyte cells. 3 PublicationsCorresponds to variant rs121918312dbSNPEnsembl.1
Natural variantiVAR_066781218R → W in CMD1HH; interferes with the assembly of Z-disks; increases stress-induced apoptosis. 1 PublicationCorresponds to variant rs397514506dbSNPEnsembl.1
Natural variantiVAR_066782258R → W Polymorphism; no functional consequences. 2 PublicationsCorresponds to variant rs117671123dbSNPEnsembl.1
Natural variantiVAR_066783300D → N.1 PublicationCorresponds to variant rs78439745dbSNPEnsembl.1
Natural variantiVAR_066784380P → S.1 PublicationCorresponds to variant rs144692954dbSNPEnsembl.1
Natural variantiVAR_048346405A → V.Corresponds to variant rs11199064dbSNPEnsembl.1
Natural variantiVAR_048347407P → L.2 PublicationsCorresponds to variant rs3858340dbSNPEnsembl.1
Natural variantiVAR_066785455E → K in CMD1HH. 1 PublicationCorresponds to variant rs397516881dbSNPEnsembl.1
Natural variantiVAR_066786462L → P in CMD1HH; interferes with the assembly of Z-disks; increases stress-induced apoptosis. 1 PublicationCorresponds to variant rs397514507dbSNPEnsembl.1
Natural variantiVAR_066787468V → M in CMD1HH. 1 Publication1
Natural variantiVAR_065480477R → H in CMD1HH. 1 PublicationCorresponds to variant rs387906876dbSNPEnsembl.1
Natural variantiVAR_066788553E → D.1 PublicationCorresponds to variant rs763530097dbSNPEnsembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF095193 mRNA. Translation: AAD16122.2.
AF127139 mRNA. Translation: AAF26839.1.
AF071218 mRNA. Translation: AAF69592.2.
AK291333 mRNA. Translation: BAF84022.1.
AL137582 mRNA. Translation: CAB70824.1.
BC006418 mRNA. Translation: AAH06418.1.
BC014656 mRNA. Translation: AAH14656.1.
BC107786 mRNA. Translation: AAI07787.1.
CCDSiCCDS7615.1.
RefSeqiNP_004272.2. NM_004281.3.
UniGeneiHs.523309.

Genome annotation databases

EnsembliENST00000369085; ENSP00000358081; ENSG00000151929.
GeneIDi9531.
KEGGihsa:9531.
UCSCiuc001lem.4. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Web resourcesi

Atlas of Genetics and Cytogenetics in Oncology and Haematology

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF095193 mRNA. Translation: AAD16122.2.
AF127139 mRNA. Translation: AAF26839.1.
AF071218 mRNA. Translation: AAF69592.2.
AK291333 mRNA. Translation: BAF84022.1.
AL137582 mRNA. Translation: CAB70824.1.
BC006418 mRNA. Translation: AAH06418.1.
BC014656 mRNA. Translation: AAH14656.1.
BC107786 mRNA. Translation: AAI07787.1.
CCDSiCCDS7615.1.
RefSeqiNP_004272.2. NM_004281.3.
UniGeneiHs.523309.

3D structure databases

ProteinModelPortaliO95817.
SMRiO95817.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi114907. 458 interactors.
DIPiDIP-41273N.
IntActiO95817. 100 interactors.
MINTiMINT-208995.
STRINGi9606.ENSP00000358081.

PTM databases

iPTMnetiO95817.
PhosphoSitePlusiO95817.

Polymorphism and mutation databases

BioMutaiBAG3.

Proteomic databases

EPDiO95817.
MaxQBiO95817.
PaxDbiO95817.
PeptideAtlasiO95817.
PRIDEiO95817.

Protocols and materials databases

DNASUi9531.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000369085; ENSP00000358081; ENSG00000151929.
GeneIDi9531.
KEGGihsa:9531.
UCSCiuc001lem.4. human.

Organism-specific databases

CTDi9531.
DisGeNETi9531.
GeneCardsiBAG3.
GeneReviewsiBAG3.
HGNCiHGNC:939. BAG3.
HPAiHPA018493.
HPA020586.
MalaCardsiBAG3.
MIMi603883. gene.
612954. phenotype.
613881. phenotype.
neXtProtiNX_O95817.
OpenTargetsiENSG00000151929.
Orphaneti154. Familial isolated dilated cardiomyopathy.
199340. Muscular dystrophy, Selcen type.
PharmGKBiPA25239.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0940. Eukaryota.
KOG4361. Eukaryota.
COG5021. LUCA.
GeneTreeiENSGT00530000063256.
HOGENOMiHOG000050234.
HOVERGENiHBG003419.
InParanoidiO95817.
KOiK09557.
OMAiVYPQLRP.
OrthoDBiEOG091G08LY.
PhylomeDBiO95817.
TreeFamiTF102013.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000151929-MONOMER.
ReactomeiR-HSA-3371453. Regulation of HSF1-mediated heat shock response.
SignaLinkiO95817.
SIGNORiO95817.

Miscellaneous databases

ChiTaRSiBAG3. human.
GeneWikiiBAG3.
GenomeRNAii9531.
PMAP-CutDBO95817.
PROiO95817.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000151929.
CleanExiHS_BAG3.
ExpressionAtlasiO95817. baseline and differential.
GenevisibleiO95817. HS.

Family and domain databases

Gene3Di1.20.58.120. 1 hit.
InterProiIPR003103. BAG_domain.
IPR001202. WW_dom.
[Graphical view]
PfamiPF02179. BAG. 1 hit.
PF00397. WW. 1 hit.
[Graphical view]
SMARTiSM00264. BAG. 1 hit.
SM00456. WW. 1 hit.
[Graphical view]
SUPFAMiSSF51045. SSF51045. 1 hit.
SSF63491. SSF63491. 1 hit.
PROSITEiPS51035. BAG. 1 hit.
PS01159. WW_DOMAIN_1. 1 hit.
PS50020. WW_DOMAIN_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiBAG3_HUMAN
AccessioniPrimary (citable) accession number: O95817
Secondary accession number(s): A8K5L8
, Q3B763, Q9NT20, Q9P120
Entry historyi
Integrated into UniProtKB/Swiss-Prot: January 11, 2001
Last sequence update: January 11, 2001
Last modified: November 30, 2016
This is version 169 of the entry and version 3 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 10
    Human chromosome 10: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.