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Heat Shock Protein Gene Table
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HSPs [HSP90, HSP70/40, HSP60/10, GroEL/ES & more]
Heme Oxygenases
Small HSPs [Crystallin, HSP20, HSP25, HSP27 & more]
Co-Chaperones & HSP-interacting Proteins
HSF
Mitochondrial HSPs [Mortalin, Grp75]
ER Stress [ERp57, ERp72, Grp94, Calnexin, Calnexin & more]
Neurodegenerative Diseases
Clusterin
More Stress & HSPs
All Stress & HSPs
Hsp70 Family
Name | Synonyms | Entrez Gene ID | SwissProt ID | Function/Structure |
APG-1 | HspA4L, Osp94 | Hsp110-subfamily | ||
APG-2 | HspA4, Irp94, Hsp70RY | Hsp110-subfamily; phosphorylated upon DNA damage, probably by ATM or ATR | ||
BIP/Grp78 | HspA5, Hsp70-5 | Constitutively expressed ER form of Hsp70, also referred to as 'immunoglobulin heavy chain binding protein' | ||
Grp75/ Mortalin | MtHsp70, HspA9, Hsp70-9, MOT, Mot-2 | Mitochondrial form of Hsp70; constitutively expressed; cooperates with Hsp60 to fold preproteins following transit across mitochondrial membrane | ||
Hsc70 | HspA8, Hsp70-8, Hsp73, Hsc71 | Constitutively expressed in most tissues; essential housekeeping gene; two isoforms due to alternative splicing | ||
Hsp70-4 | Hsp70L1 HspA14 | Hsp70-like protein 1; cloned from human dendritic cells | ||
Hsp70B | HspA7 | Tightly linked to Hsp70B' locus | ||
Hsp70B' | HspA6, Hsp70-6 | Strictly stress-inducible; in unstressed conditions it is expressed only in certain blood cells (dendritic cells, monocytes and NK cells) | ||
Hsp70-Hom | HspA1L, Hum70t, Hsp70-1t | Constitutive, testis-specific expression; locus adjacent to Hsp72 and Hsp70-2 | ||
Hsp72 | HspA1A, Hsp70-1a, Hsp70-1 | Stress-inducible; highly expressed in various cancers; corrlelated with increased proliferation, metastasis and poor outcome in breast cancer | ||
Hsp72 | HspA1B, Hsp70-1b, Hsp70-1 | Stress-inducible; identical in protein sequence to HspA1A above, but transcribed from an adjacent locus | ||
Hsp110 | Hsp105, HspH1 | Hsp110-subfamily; acts as nucleotide exchange factor for Hsp70 | ||
HspA2 | Hsp70-2 | Constitutively expressed at high levels in brain and testis; role in spermatogenesis; upregulated in a subset of breast cancers and has growth and survival promoting effects in cancer cells | ||
HYOU1 | Hypoxia upregulated 1, Grp170, ORP 150 | Induced by hypoxia, found in the ER, upregulated in tumors; protein with alternative translation start site found in the cytoplasm |
Hsp70/Hsp40 Associated Co-chaperones & Others
Name | Synonyms | Entrez Gene ID | SwissProt ID | Function/Structure |
Bag-1 | HAP | BAG proteins compete with Hip for binding to the Hsc70/Hsp70 ATPase domain and promote substrate release; two splice variants | ||
Bag-2 | NA | Associates with CHIP and inhibits/regulates its activity by inhibiting the interaction between CHIP and its cognate ubiquitin-conjugating enzyme, UbcH5a; phosphorylated at Ser20 by MAPKAPK-2 | ||
Bag-3 | BIS | Binds ATPase domain of Hsp70/Hsc70, inhibits chaperone activity | ||
Bag-4 | SODD | Inhibits Hsp70/Hsc70; prevents constitutive TNFRSF1A signaling; cytosolic | ||
Bag-5 | NA | Negative regulator of Hsp70; two alternative splice forms | ||
BAP | SIL1 | ER localized protein that is associated with BiP/Grp94 and serves as a nucleotide exchange factor for it; mutations associated with Marinesco- Sjogren syndrome; two splice variants that encode the same protein | ||
CHIP | STUB1, UBOX1, HSPABP2 | Carboxyl terminus of Hsp70 Interacting Protein; an Hsp70-associated E3 ubiquitin ligase that targets chaperone substrates to the proteasome; inhibited by Hip and BAG-2; interacts with HSF1 upon heat shock; substrates include AR, Tau, Smad1/4, nNOS and Cu/Zn-SOD | ||
Hip | HspBP1 | Nucleotide exchange factor for Hsp70; inhibits the ubiquitin ligase activity of CHIP; an anti-apoptotic protein and substrate of Granzyme B | ||
Hop | STIP1, STI1 | Hsp70/Hsp90-organizing protein; Stress-induced Phosphoprotein 1; binds EEVD motifs at the C-termini of Hsp70 and Hsp90; also binds Cdc37; normally found in the cytoplasm, but also seen in the nucleus depending on cell cycle/CDK activity |
Hsp40/DnaJ Family
Name | Synonyms | Entrez Gene ID | SwissProt ID | Function/Structure |
CSP | Cysteine String Protein, DnaJC5 | Secretory vesicle protein involved in regulated exocytosis; plays a role in CFTR biogenesis and trafficking; interacts with the inactive GDP- bound form of Galpha(s) and promotes GDP/GTP exchange; guanine nucleotide exchange activity regulated by Hsc70 and SGT | ||
CSP-beta | DnaJC5B | Testis specific expression; palmitoylated | ||
CSP-gamma | DnaJC5G | Testis specific expression; palmitoylated | ||
DnaJA4 | NA | Hsp40 homolog | ||
DnaJA5 | NA | Hsp40 homolog; three splice variants; expressed in brain, placenta, kidney, and pancreas | ||
DnaJB12 | NA | Possibly membrane-associated | ||
DnaJB13 | TSARG6 | Expressed specifically in adult testis, implicated in apoptosis in spermatogenic cells | ||
DnaJB14 | NA | Possibly membrane-associated; two splice variants | ||
DnaJB8 | NA | Hsp40 homolog | ||
DnaJC11 | FLJ10737 | Candidate tumor suppressor gene of neuroblastoma; found in mitochondria | ||
DnaJC16 | NA | Possibly type IV single-pass membrane protein; two splice variant | ||
DnaJC17 | NA | Hsp40 homolog; contains RNA recognition motif | ||
DnaJC18 | NA | Possibly single-pass membrane protein | ||
DnaJC19 | TIM14 | Mitochondrial protein, similar in sequence to yeast Tim14 which is involved in mitochondrial protein import; mutated in DCMA syndrome (dilated cardiomyopathy with ataxia) | ||
DnaJC4 | HSPF2; MCG18 | Possibly single-pass membrane protein | ||
DnaJC6 | DJC6 | Involved in uncoating of clathrin coated vesicles; interacts with Hsc70; three splice variants | ||
DnaJC7 | TPR2; TTC2 | Interacts with GAP domain of NF1 | ||
DnaJC8 | SPF31 | Splicing protein spf31 | ||
DRIP78 | HDJ3; DnaJC14 | May regulate trafficking of GPCR's including Angiotensin II and Dopamine Receptor; may play a role as a chaperone in the assembly of G-beta/gamma subunits | ||
ERdj3 | DnaJB11, ERj3, HEDJ; ABBP-2 | ER localized, expression induced by ER stress; stimulates BiP ATPase activity | ||
ERdj5 | JPDI; DnaJC10 | ER localized, expression induced by ER stress (UPR); also a member of the Protein Disulfide Isomerase family | ||
Hdj2 | DnaJA1, DJ2; DJA1; HSDJ; HSJ2 | Farnesylated; interacts with Hsc70; two splice forms, one nuclear, one expressed throughout the cell | ||
HdjC9 | JDD1; DnaJC9 | Hsp40 homolog | ||
Hlj1 | DnaJB4, DjB4; DnaJW | Identified in human liver | ||
HSC3 | DnaJB7 | ER localized, expression induced by ER stress (UPR); also a member of the Protein Disulfide Isomerase family | ||
Hsc40 | DnaJB5 | Constitutive Hsp40 isoform | ||
HscB | DnaJC20, Jac1, Hsc20 | Homolog of E. coli HscB, which along with the E. coli Hsp70 homolog HscA forms part of a specialized system for synthesizing iron-sulfur proteins; candidate gene in hereditary ataxia syndromes | ||
Hsj1 | DnaJB2, HSPF3 | Neuronal tissue-specific; two isoforms (Hsj1a and Hsj1b) | ||
Hsp40/Hdj1 | DnaJB1, HSPF1 | Enhances ATPase activity of Hsp70 family members | ||
Htj1/ERdj1 | DNAJC1, DNAJL1, MTJ1 | Enriched in microsomes and nucleus; stimulates BiP ATPase activity; involved in transport of BiP to the cell surface | ||
JDP1 | DnaJC12 | An estrogen target gene, highly expressed in ER positive breast cancer; two isoforms; conserved down to Drosophila | ||
MCJ | HSD18, DNAJD1, DNAJC15 | Methylation controlled J protein (MCJ) is a type II transmembrane cochaperone localized in the Golgi network and present only in vertebrates | ||
Mdg1/ERdj4 | DnaJB9 | ER localized, expression induced by UPR; expression associated with low metastatic potential; stimulates BiP ATPase activity | ||
MPP11 | MIDA1, DnaJC2, Zrf1, Zrf2 | Localized to nucleus; ubiquitously expressed | ||
Mrj | DnaJB6, Hsj2; Msj1 | Enriched in the CNS; two isoforms as a result of alternative splicing; one nuclear, one expressed throughout the cell; binds and regulates NFAT | ||
P58(IPK) | DnaJC3, PRKRI; | Binds and inactivates PKR; induced by ER stress, binds and inactivates PERK | ||
Rdj2 | DnaJA2, DJA2 | Hsc70 co-chaperone; possibly membrane-associated and farnesylated | ||
RME-8 | DnaJC13 | Functions in endosomal trafficking; human homolog of Receptor-mediated endocytosis 8 (RME8) in C. elegans; widely expressed, binds Hsc70 | ||
Tid1 | hTid-1, DnaJA3 | Mitochondrial; modulates apoptotic signal transduction, cytochrome c release, and caspase 3 activation; two splice variants |
Hsp60/Hsp10 Family
Name | Synonyms | Entrez Gene ID | SwissProt ID | Function/Structure |
CCT2 | chaperonin containing TCP1 subunit 2 (beta), TCP1-beta, CCTB, CCT-beta; | Component of hetero-oligomeric TRiC complex | ||
CCT3 | chaperonin containing TCP1 subunit 3 (gamma), CCTG, CCT-gamma, TRIC5, TCP1-gamma | Component of hetero-oligomeric TRiC complex; three alternative splice forms | ||
CCT4 | chaperonin containing TCP1 subunit 4 (delta), SRB, CCTd | Component of hetero-oligomeric TRiC complex | ||
CCT5 | chaperonin containing TCP1 subunit 5 (epsilon), CCTE, CCT-epsilon, TCP1-epsilon | Component of hetero-oligomeric TRiC complex; mutation associated with autosomal recessive mutilating sensory neuropathy with spastic paraplegia; expression up-regulated in p53-mutated tumors | ||
CCT6A | chaperonin containing TCP1 subunit 6A (zeta 1), CCT6, CCTz, HTR3, TCPZ, TCP20, MoDP-2, TTCP20, CCT-zeta, CCT-zeta1, TCP1-zeta | Component of hetero-oligomeric TRiC complex; ionizing radiation results in enhanced expression in radioresistant cancer cells compared to radiosensitive cells; two alternative splice forms | ||
CCT6B | chaperonin containing TCP1 subunit 6B (zeta 2), CCT-zeta2; TCP1-zeta2 | Component of hetero-oligomeric TRiC complex; tesis specific expression | ||
CCT7 | chaperonin containing TCP1 subunit 7 (eta), HIV-1 Nef interacting protein ( Nip7-1), TCP1-eta, Ccth, CCT-Eta | Component of hetero-oligomeric TRiC complex; two alternative splice forms | ||
Hsp10 | CPN10, GroES, HSPE1 | Closely linked to the Hsp60 gene (HspD1); forms chaperonin 'cap' structure | ||
Hsp60 | HspD1, CPN60, GroEL, HSP65, SPG13, HuCHA60 | Mitochondrial protein essential for folding and assembly of newly imported proteins; also a signaling molecule in the innate immune system; mutations associated with autosomal recessive spastic paraplegia | ||
TCP1 | CCT1, CCT-alpha, TCP1-alpha | Member of the chaperonin containing TCP1 complex (CCT), also known as the TCP1 ring complex (TRiC), consisting of two identical stacked rings, each containing eight different proteins; the complex folds various proteins, including actin and tubulin, in an ATP-dependent manner; two alternative splice forms;unlike Hsp60, no known associated Hsp10/GroES cofactor |
Hsp90 Family
Name | Synonyms | Entrez Gene ID | SwissProt ID | Function/Structure |
Grp94 | Hsp90B1, Gp96 | ER form; glucose regulated and induced by glucose starvation; participates in protein folding and assembly, protein secretion, protecting cells from apoptosis, and mediating immunogenicity; C-terminal sequence KDEL mediates | ||
Hsp90 alpha | Hsp90AA1, Hsp86, Hsp89 | Cytosolic form, induced by elevated temperature; ATPase activity; three splice variants | ||
Hsp90 alpha | Hsp90AA2 | Cytosolic form, induced by elevated temperature | ||
Hsp90 beta | Hsp90AB1 | Cytosolic form, constitutively expressed; ATPase activity; three splice variants | ||
TRAP1 | Hsp75 | Mitochondrial form; has ATPase activity that is inhibited by both geldanamycin and radicicol; highly conserved through evolution; phosphorylation by PINK1 prevents oxidative-stress-induced apoptosis; four splice variants | ||
Hsp90 Associated Co-chaperones & Others
Name | Synonyms | Entrez Gene ID | SwissProt ID | Function/Structure |
Aha1 | Activator of Hsp90 ATPase homolog 1 | Stimulates the inherent ATPase activity of Hsp90 | ||
Cdc37 | NA | A chaperone that binds Hsp90 and is required for the activity of numerous protein kinases | ||
Hop | STIP1, STI1 | Golgi; Hsp70/Hsp90-organizing protein; Stress-induced Phosphoprotein 1; binds EEVD motifs at the C-termini of Hsp70 and Hsp90; also binds Cdc37; normally found in the cytoplasm, but also seen in the nucleus depending on cell cycle/CDK activity | ||
p23 | TEBP, PGE Synthase 3 | Binds to telomerase and progesterone receptor; also functions as a cytosolic prostaglandin E2 synthase; phosphorylated at Ser113, 118, 148 and 151 and acetylated at Lys33 |
Small Hsp Family (sHsps)
Name | Synonyms | Entrez Gene ID | SwissProt ID | Function/Structure |
Crystallin, alpha A | CRYAA, CRYA1, HSPB4 | Expression restricted to the lens | ||
Crystallin, alpha B | CRYAB, CRYA2, HSPB5 | Broad tissue expression; elevated expression in many neurological diseases; a missense mutation associated with a desmin-related myopathy | ||
Heat shock 22kDa protein 8 | H11, E2IG1, HSP22, HSPB8 | Charcot-Marie-Tooth disease type 2L; Hereditary motor neuropathy type II | ||
heat shock 27kDa protein 1 | Hsp27, Hsp25, HSPB1 | Charcot-Marie-Tooth disease, axonal, type 2F; distal hereditary motor neuropathy | ||
heat shock 27kDa protein 2 | HSPB2, MKBP | Associates with myotonic dystrophy protein kinase (DMPK) | ||
heat shock 27kDa protein 3 | HSPB3, HSPL27 | Inhibitor of actiin polymerization | ||
heat shock 27kDa protein family, member 7 (cardiovascular) | cvHSP, HspB7 | Bovine βH-crystallin | ||
heat shock protein, alpha-crystallin-related, B6 | HSPB6, Hsp20 | Structural component of eye lens | ||
heat shock protein, alpha-crystallin-related, B9 | HSPB9, CT51 | Testis specific | ||
outer dense fiber of sperm tails 1 | HSPB10, ODFP, SODF, ODF1 | Component of outer dense fibers of spermatozoa; testis specific expression | ||
Beta/gamma Crystallin family
Name | Synonyms | Entrez Gene ID | SwissProt ID | Function/Structure |
Crystallin, beta A1 | CRYBA1 | Mutation causes the autosomal dominant disease 'zonular cataract with sutural opacities'; member of the acidic group of beta crystallins; beta- crystallins form aggregates of different sizes and are able to self-associate to form dimers or to form heterodimers with other beta-crystallins | ||
Crystallin, beta A2 | CRYBA2 | Member of the acidic group of beta crystallins | ||
Crystallin, beta A4 | CRYBA4 | Member of the acidic group of beta crystallins; part of a gene cluster with beta B1, beta B2, and beta B3; mutations linked to cataractogenesis and microphthalmia | ||
Crystallin, beta B1 | CRYBB1 | Member of the basic group of beta crystallins | ||
Crystallin, beta B2 | CRYBB2 | Member of the basic group of beta crystallins; mutation found to cause type 2 cerulean cataracts | ||
Crystallin, beta B3 | CRYBB3 | Member of the basic group of beta crystallins | ||
Crystallin, gamma A | CRYGA, CRYG1, CRYG5 | Part of a gene cluster with gamma B, C and D, and the pseudogenes gamma-E, F and G; gamma-crystallins are monomeric proteins and have been implicated in cataract formation | ||
Crystallin, gamma B | CRYGB, CRYG2 | Part of a gene cluster with gamma A, C and D, and the pseudogenes gamma-E, F and G; gamma-crystallins are monomeric proteins and have been implicated in cataract formation | ||
Crystallin, gamma C | CRYGC, CRYG3, CCL | Part of a gene cluster with gamma A, B, and D, and the pseudogenes gamma-E, F and G; gamma-crystallins are monomeric proteins and have been | ||
Crystallin, gamma D | CRYGD, CRYG4, CCP, PCC, CACA, CCA3 | Part of a gene cluster with gamma A, B, and C, and the pseudogenes gamma-E, F and G; gamma-crystallins are monomeric proteins and have been implicated in cataract formation | ||
Crystallin, gamma N | CRYGN | beta-gamma hybrid crystallin; expressed in retina and lens nuclear fibers in rodents | ||
Crystallin, gamma S | CRYGS, CRYG8 | The most significant gamma-crystallin in adult eye lens tissue |
Other Hsps and Chaperones
Name | Synonyms | Entrez Gene ID | SwissProt ID | Function/Structure |
Calnexin | CNX, P90 | Calcium-binding ER protein that interacts transiently with newly synthesized N-linked glycoproteins, facilitating protein folding and assembly; it may also play a central role in the quality control of protein folding by retaining incorrectly folded protein subunits within the ER for degradation | ||
Calreticulin | CRTC, ERp60, grp60 | Calcium-binding ER protein; also found in the nucleus; can bind and inhibit nuclear hormone receptors | ||
CHIP | STUB1, UBOX1, HspABP2 | E3 ubiquitin ligase; co-chaperone that associates with Hsp70 and Hsp90 | ||
HO-1 | heme oxygenase (decyclizing) 1, HMOX1, Hsp32 | Highly inducible by heavy metals, endotoxin, oxidizing agents, UVA; cleaves heme ring at the alpha methene bridge to form biliverdin | ||
HO-2 | heme oxygenase (decyclizing) 2, HMOX2 | Non-inducible; cleaves heme ring at the alpha methene bridge to form biliverdin | ||
HSF-1 | HSTF1 | Regulates the program of heat shock gene expression. Expression repressed by Hsp90; positively and negatively regulated by phosphorylation; modified by SUMO-1 and SUMO-2 in a stress-inducible manner | ||
HSF-2 | HSTF2 | Regulates the program of heat shock gene expression | ||
Hsp47 | SerpinH1, Colligin, Gp46 | ER-localized member of the serpin family of serine protease inhibitors; expression induced by heat shock; binds collagen and thought to be a chaperone involved in the maturation of collagen; autoantibodies found in patients with rheumatoid arthritis | ||
KDELR1 | ERD2 | Family of seven-transmembrane receptors that mediate the retention of proteins with the sequence lys-asp-glu-leu (KDEL), such as Grp78 and Grp94, in the lumen of the ER | ||
KDELR2 | ERD2.2 | Two alternative splice forms | ||
KDELR3 | ERD2L3 | Two alternative splice forms | ||
UGGT | UDP-glucose:glycoprotein glucosyltransferase, HUGT1, UGCGL1, GT, UGT1, UGTR | Recognizes glycoproteins with minor folding defects; reglucosylates single N-glycans near the misfolded part of the protein, thus providing quality control for protein folding in the endoplasmic reticulum; reglucosylated proteins are recognized by calreticulin for recycling to the endoplasmic reticulum and refolding or degradation | ||
Protein Disulfide Isomerase (PDI) Family
Name | Synonyms | Entrez Gene ID | SwissProt ID | Function/Structure |
ERdj5 | JPDI; DnaJC10 | Expression induced by ER stress; also a member of the DnaJ family | ||
ERp18 | TXNDC12, AGR1, ERp19 | Member of the thioredoxin superfamily; expressed in ER lumen | ||
ERp27 | NA | Interacts with PDIA3; does not contain a CXXC active site motif indicating that it is a catalytically redox-inactive member of the protein disulfide isomerase family | ||
ERp29 | ERp28, ERp31, PDI-DB | Similar in sequence to the PDI family, but lacks a thioredoxin motif, suggesting it does not function as a disulfide isomerase; the protein dimerizes and is thought to play a role in the processing of secretory proteins within the ER; two alternative splice forms | ||
ERp44 | TXNDC4 | Induced by ER stress | ||
ERp46 | TXNDC5 | Highly expressed in endothelial cells, where it is induced by and protects against hypoxia; contains 3 thioredoxin domains; two isoforms | ||
ERp57 | Grp58, PDIA3 | ER protein with protein disulfide isomerase activity that interacts with the lectin chaperones calreticulin and calnexin to modulate the folding of newly synthesized glycoproteins | ||
ERp72 | PDIA4, ERp70 | Catalyzes rearrangement of disulfide bonds | ||
P5 | PDIA6, ERP5, TXNDC7 | Can also function on the cell surface | ||
PDI | PDIA1, P4HB | Catalyzes S-S bond formation, breakage, and rearrangement in nascent (ER lumen) and cell surface proteins | ||
PDILT |
| Testis-specific expression | ||
PDIP | PDIA2 | Catalyzes S-S bond rearrangement; expressed in ER lumen; high expression in pancreas | ||
PDIR | PDIA5 | Contains 3 thioredoxin domains | ||
TMX | TXNDC1 | Contains one thioredoxin domain and a putative transmembrane domain | ||
TMX2 | TXNDC14 | Contains one thioredoxin domain and a putative transmembrane domain | ||
TMX3 | TXNDC10 | Contains one thioredoxin domain and a putative transmembrane domain | ||
TMX4 | TXNDC13 | Contains one thioredoxin domain and a putative transmembrane domain | ||
