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Protein

BAG family molecular chaperone regulator 3

Gene

BAG3

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

Functioni

Co-chaperone for HSP70 and HSC70 chaperone proteins. Acts as a nucleotide-exchange factor (NEF) promoting the release of ADP from the HSP70 and HSC70 proteins thereby triggering client/substrate protein release. Nucleotide release is mediated via its binding to the nucleotide-binding domain (NBD) of HSPA8/HSC70 where as the substrate release is mediated via its binding to the substrate-binding domain (SBD) of HSPA8/HSC70 (PubMed:9873016, PubMed:27474739). Has anti-apoptotic activity (PubMed:10597216). Plays a role in the HSF1 nucleocytoplasmic transport (PubMed:26159920).5 Publications

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionChaperone
Biological processApoptosis

Enzyme and pathway databases

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

EuPathDBiHostDB:ENSG00000151929.9
HGNCiHGNC:939 BAG3
MIMi603883 gene
neXtProtiNX_O95817

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Cytoplasm, Nucleus

Pathology & Biotechi

Involvement in diseasei

Myopathy, myofibrillar, 6 (MFM6)3 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 disk 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 dbSNP:rs121918312EnsemblClinVar.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 dbSNP:rs387906874EnsemblClinVar.1
Natural variantiVAR_066781218R → W in CMD1HH; interferes with the assembly of Z-disks; increases stress-induced apoptosis. 1 PublicationCorresponds to variant dbSNP:rs397514506EnsemblClinVar.1
Natural variantiVAR_066785455E → K in CMD1HH. 1 PublicationCorresponds to variant dbSNP:rs397516881EnsemblClinVar.1
Natural variantiVAR_066786462L → P in CMD1HH; interferes with the assembly of Z-disks; increases stress-induced apoptosis. 1 PublicationCorresponds to variant dbSNP:rs397514507EnsemblClinVar.1
Natural variantiVAR_066787468V → M in CMD1HH. 1 Publication1
Natural variantiVAR_065480477R → H in CMD1HH. 1 PublicationCorresponds to variant dbSNP:rs387906876EnsemblClinVar.1

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi480 – 481RR → AA: Significant loss of interaction with HSPA8. 1 Publication2

Keywords - Diseasei

Cardiomyopathy, Disease mutation, Myofibrillar myopathy

Organism-specific databases

DisGeNETi9531
GeneReviewsiBAG3
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); alternateCombined sources
Cross-linki445Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2); alternateCombined sources

Keywords - PTMi

Acetylation, Isopeptide bond, Methylation, Phosphoprotein, Ubl conjugation

Proteomic databases

EPDiO95817
MaxQBiO95817
PaxDbiO95817
PeptideAtlasiO95817
PRIDEiO95817
ProteomicsDBi51065

PTM databases

iPTMnetiO95817
PhosphoSitePlusiO95817

Miscellaneous databases

PMAP-CutDBiO95817

Expressioni

Gene expression databases

BgeeiENSG00000151929 Expressed in 232 organ(s), highest expression level in deltoid
CleanExiHS_BAG3
ExpressionAtlasiO95817 baseline and differential
GenevisibleiO95817 HS

Organism-specific databases

HPAiHPA018493
HPA020586

Interactioni

Subunit structurei

Binds to the ATPase domain of HSP70/HSC70 chaperones (PubMed:9873016). Interacts with BCL2 (PubMed:10597216). Interacts with phospholipase C-gamma proteins (PubMed:10980614). Interacts with DNAJB6 (PubMed:22366786). Interacts (via BAG domain) with HSF1; this interaction occurs in normal and heat-shocked cells promoting HSF1 nucleocytoplasmic shuttling (PubMed:26159920). Interacts with HSPA8 (via NBD) (PubMed:27474739, PubMed:24318877). Interacts with HSPA1A (via NBD) and HSPA1B (via NBD) (PubMed:24318877). Interacts (via WW domain 1) with SYNPO2 (via PPPY motif) (PubMed:23434281). Interacts with HSPB8 (PubMed:28144995).9 Publications

Binary interactionsi

GO - Molecular functioni

Protein-protein interaction databases

BioGridi114907, 487 interactors
CORUMiO95817
DIPiDIP-41273N
IntActiO95817, 134 interactors
MINTiO95817
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

Keywords - Domaini

Repeat

Phylogenomic databases

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

Family and domain databases

CDDicd00201 WW, 1 hit
Gene3Di1.20.58.120, 1 hit
InterProiView protein in InterPro
IPR039773 BAG_chaperone_regulator
IPR036533 BAG_dom_sf
IPR003103 BAG_domain
IPR001202 WW_dom
IPR036020 WW_dom_sf
PANTHERiPTHR12329 PTHR12329, 1 hit
PfamiView protein in Pfam
PF02179 BAG, 1 hit
PF00397 WW, 1 hit
SMARTiView protein in SMART
SM00264 BAG, 1 hit
SM00456 WW, 1 hit
SUPFAMiSSF51045 SSF51045, 1 hit
SSF63491 SSF63491, 1 hit
PROSITEiView protein in PROSITE
PS51035 BAG, 1 hit
PS01159 WW_DOMAIN_1, 1 hit
PS50020 WW_DOMAIN_2, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

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

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All

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

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
C9JFK9C9JFK9_HUMAN
BAG family molecular chaperone regu...
BAG3
325Annotation score:

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 → Q1 PublicationCorresponds to variant dbSNP:rs35434411EnsemblClinVar.1
Natural variantiVAR_06547971R → W in CMD1HH. 1 PublicationCorresponds to variant dbSNP:rs387906874EnsemblClinVar.1
Natural variantiVAR_06677777P → L1 PublicationCorresponds to variant dbSNP:rs141355480EnsemblClinVar.1
Natural variantiVAR_06677894I → F1 PublicationCorresponds to variant dbSNP:rs145393807EnsemblClinVar.1
Natural variantiVAR_066779115P → S1 PublicationCorresponds to variant dbSNP:rs774241343Ensembl.1
Natural variantiVAR_048345151C → R2 PublicationsCorresponds to variant dbSNP:rs2234962EnsemblClinVar.1
Natural variantiVAR_066780155A → T1 PublicationCorresponds to variant dbSNP:rs61756328EnsemblClinVar.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 dbSNP:rs121918312EnsemblClinVar.1
Natural variantiVAR_066781218R → W in CMD1HH; interferes with the assembly of Z-disks; increases stress-induced apoptosis. 1 PublicationCorresponds to variant dbSNP:rs397514506EnsemblClinVar.1
Natural variantiVAR_066782258R → W Polymorphism; no functional consequences. 2 PublicationsCorresponds to variant dbSNP:rs117671123EnsemblClinVar.1
Natural variantiVAR_066783300D → N1 PublicationCorresponds to variant dbSNP:rs78439745EnsemblClinVar.1
Natural variantiVAR_066784380P → S1 PublicationCorresponds to variant dbSNP:rs144692954EnsemblClinVar.1
Natural variantiVAR_048346405A → V. Corresponds to variant dbSNP:rs11199064EnsemblClinVar.1
Natural variantiVAR_048347407P → L2 PublicationsCorresponds to variant dbSNP:rs3858340EnsemblClinVar.1
Natural variantiVAR_066785455E → K in CMD1HH. 1 PublicationCorresponds to variant dbSNP:rs397516881EnsemblClinVar.1
Natural variantiVAR_066786462L → P in CMD1HH; interferes with the assembly of Z-disks; increases stress-induced apoptosis. 1 PublicationCorresponds to variant dbSNP:rs397514507EnsemblClinVar.1
Natural variantiVAR_066787468V → M in CMD1HH. 1 Publication1
Natural variantiVAR_065480477R → H in CMD1HH. 1 PublicationCorresponds to variant dbSNP:rs387906876EnsemblClinVar.1
Natural variantiVAR_066788553E → D1 PublicationCorresponds to variant dbSNP:rs763530097EnsemblClinVar.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

Similar proteinsi

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, 487 interactors
CORUMiO95817
DIPiDIP-41273N
IntActiO95817, 134 interactors
MINTiO95817
STRINGi9606.ENSP00000358081

PTM databases

iPTMnetiO95817
PhosphoSitePlusiO95817

Polymorphism and mutation databases

BioMutaiBAG3

Proteomic databases

EPDiO95817
MaxQBiO95817
PaxDbiO95817
PeptideAtlasiO95817
PRIDEiO95817
ProteomicsDBi51065

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
EuPathDBiHostDB:ENSG00000151929.9
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
OMAiQTGWPFF
OrthoDBiEOG091G08LY
PhylomeDBiO95817
TreeFamiTF102013

Enzyme and pathway databases

ReactomeiR-HSA-3371453 Regulation of HSF1-mediated heat shock response
SignaLinkiO95817
SIGNORiO95817

Miscellaneous databases

ChiTaRSiBAG3 human
GeneWikiiBAG3
GenomeRNAii9531
PMAP-CutDBiO95817
PROiPR:O95817
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000151929 Expressed in 232 organ(s), highest expression level in deltoid
CleanExiHS_BAG3
ExpressionAtlasiO95817 baseline and differential
GenevisibleiO95817 HS

Family and domain databases

CDDicd00201 WW, 1 hit
Gene3Di1.20.58.120, 1 hit
InterProiView protein in InterPro
IPR039773 BAG_chaperone_regulator
IPR036533 BAG_dom_sf
IPR003103 BAG_domain
IPR001202 WW_dom
IPR036020 WW_dom_sf
PANTHERiPTHR12329 PTHR12329, 1 hit
PfamiView protein in Pfam
PF02179 BAG, 1 hit
PF00397 WW, 1 hit
SMARTiView protein in SMART
SM00264 BAG, 1 hit
SM00456 WW, 1 hit
SUPFAMiSSF51045 SSF51045, 1 hit
SSF63491 SSF63491, 1 hit
PROSITEiView protein in PROSITE
PS51035 BAG, 1 hit
PS01159 WW_DOMAIN_1, 1 hit
PS50020 WW_DOMAIN_2, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiBAG3_HUMAN
AccessioniPrimary (citable) accession number: O95817
Secondary accession number(s): A8K5L8
, Q3B763, Q9NT20, Q9P120
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 11, 2001
Last sequence update: January 11, 2001
Last modified: November 7, 2018
This is version 188 of the entry and version 3 of the sequence. See 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 entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  2. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  3. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  4. Human chromosome 10
    Human chromosome 10: entries, gene names and cross-references to MIM
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Main funding by: National Institutes of Health

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