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Heat Shock Protein Gene Table

Hsp70 Family

Name Synonyms Entrez Gene ID SwissProt ID Function/Structure
APG-1 HspA4L, Osp94 22824 O95757 Hsp110-subfamily
APG-2 HspA4, Irp94, Hsp70RY 3308 P34932 Hsp110-subfamily; phosphorylated upon DNA damage, probably by ATM or ATR
BIP/Grp78 HspA5, Hsp70-5 3309 P11021 Constitutively expressed ER form of Hsp70, also referred to as 'immunoglobulin heavy chain binding protein'
Grp75/ Mortalin MtHsp70, HspA9, Hsp70-9, MOT, Mot-2 3313 P38646 Mitochondrial form of Hsp70; constitutively expressed; cooperates with Hsp60 to fold preproteins following transit across mitochondrial membrane 
Hsc70 HspA8, Hsp70-8, Hsp73, Hsc71 3312 P11142 Constitutively expressed in most tissues; essential housekeeping gene; two isoforms due to alternative splicing
Hsp70-4 Hsp70L1 HspA14 51182 Q0VDF9 Hsp70-like protein 1; cloned from human dendritic cells
Hsp70B HspA7 3311 P48741 Tightly linked to Hsp70B' locus
Hsp70B' HspA6, Hsp70-6 3310 P17066 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 3305 P34931 Constitutive, testis-specific expression; locus adjacent to Hsp72 and Hsp70-2
Hsp72 HspA1A, Hsp70-1a, Hsp70-1 3303 P08107 Stress-inducible; highly expressed in various cancers; corrlelated with increased proliferation, metastasis and poor outcome in breast cancer
Hsp72 HspA1B, Hsp70-1b, Hsp70-1 3304 P08107 Stress-inducible; identical in protein sequence to HspA1A above, but transcribed from an adjacent locus
Hsp110 Hsp105, HspH1 10808 Q92598 Hsp110-subfamily; acts as nucleotide exchange factor for Hsp70
HspA2 Hsp70-2 3306 P54652 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 10525 Q9Y4L1 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 573 Q99933 BAG proteins compete with Hip for binding to the Hsc70/Hsp70 ATPase domain and promote substrate release; two splice variants
Bag-2 NA 9532 O95816 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 9531 O95817 Binds ATPase domain of Hsp70/Hsc70, inhibits chaperone activity
Bag-4 SODD 9530 O95429 Inhibits Hsp70/Hsc70; prevents constitutive TNFRSF1A signaling; cytosolic
Bag-5 NA 9529 Q9UL15 Negative regulator of Hsp70; two alternative splice forms
BAP SIL1 64374 Q9H173 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 10273 Q9UNE7 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  23640 Q9NZL4 Nucleotide exchange factor for Hsp70; inhibits the ubiquitin ligase activity of CHIP; an anti-apoptotic protein and substrate of Granzyme B
Hop STIP1, STI1 10963 P31948 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 80331 Q9H3Z4 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 85479 Q9UF47 Testis specific expression; palmitoylated
CSP-gamma DnaJC5G 285126 Q8N7S2 Testis specific expression; palmitoylated
DnaJA4 NA 55466 Q8WW22 Hsp40 homolog
DnaJA5 NA 134218 Q5F1R6 Hsp40 homolog; three splice variants; expressed in brain, placenta, kidney, and pancreas
DnaJB12 NA 54788 Q9NXW2 Possibly membrane-associated
DnaJB13 TSARG6 374407 P59910 Expressed specifically in adult testis, implicated in apoptosis in spermatogenic cells
DnaJB14 NA 79982 Q8TBM8 Possibly membrane-associated; two splice variants
DnaJB8 NA 165721 Q8NHS0 Hsp40 homolog
DnaJC11 FLJ10737 55735 Q9NVH1 Candidate tumor suppressor gene of neuroblastoma; found in mitochondria
DnaJC16 NA 23341 Q9Y2G8 Possibly type IV single-pass membrane protein; two splice variant
DnaJC17 NA 55192 Q9NVM6 Hsp40 homolog; contains RNA recognition motif
DnaJC18 NA 202052 Q9H819 Possibly single-pass membrane protein
DnaJC19 TIM14 131118 Q96DA6 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 3338 Q9NNZ3 Possibly single-pass membrane protein
DnaJC6 DJC6 9829 O75061 Involved in uncoating of clathrin coated vesicles; interacts with Hsc70; three splice variants
DnaJC7 TPR2; TTC2 7266 Q99615 Interacts with GAP domain of NF1
DnaJC8 SPF31 22826 O75937 Splicing protein spf31
DRIP78 HDJ3; DnaJC14 85406 Q6Y2X3 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 51726 Q9UBS4 ER localized, expression induced by ER stress; stimulates BiP ATPase activity
ERdj5 JPDI; DnaJC10 54431 Q8IXB1 ER localized, expression induced by ER stress (UPR); also a member of the Protein Disulfide Isomerase family
Hdj2 DnaJA1, DJ2; DJA1; HSDJ; HSJ2 3301 P31689 Farnesylated; interacts with Hsc70; two splice forms, one nuclear, one expressed throughout the cell
HdjC9 JDD1; DnaJC9 23234 Q8WXX5 Hsp40 homolog
Hlj1 DnaJB4, DjB4; DnaJW 11080 Q9UDY4 Identified in human liver
HSC3 DnaJB7 150353 Q7Z6W7 ER localized, expression induced by ER stress (UPR); also a member of the Protein Disulfide Isomerase family
Hsc40 DnaJB5 25822 O75953 Constitutive Hsp40 isoform
HscB DnaJC20, Jac1, Hsc20 150274 Q8IWL3 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 3300 P25686 Neuronal tissue-specific; two isoforms (Hsj1a and Hsj1b)
Hsp40/Hdj1 DnaJB1, HSPF1 3337 P25685 Enhances ATPase activity of Hsp70 family members
Htj1/ERdj1 DNAJC1, DNAJL1, MTJ1 64215 Q96KC8 Enriched in microsomes and nucleus; stimulates BiP ATPase activity; involved in transport of BiP to the cell surface
JDP1 DnaJC12 56521 Q9UKB3 An estrogen target gene, highly expressed in ER positive breast cancer;  two isoforms; conserved down to Drosophila
MCJ HSD18, DNAJD1, DNAJC15 29103 Q9Y5T4 Methylation controlled J protein (MCJ) is a type II transmembrane cochaperone localized in the Golgi network and present only in vertebrates
Mdg1/ERdj4 DnaJB9 4189 Q9UBS3 ER localized, expression induced by UPR; expression associated with low metastatic potential; stimulates BiP ATPase activity
MPP11 MIDA1, DnaJC2, Zrf1, Zrf2 22791 P54103 Localized to nucleus; ubiquitously expressed
Mrj DnaJB6,  Hsj2; Msj1 10049 O75190 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; 5611 Q13217 Binds and inactivates PKR; induced by ER stress, binds and inactivates PERK
Rdj2 DnaJA2, DJA2 10294 O60884 Hsc70 co-chaperone; possibly membrane-associated and farnesylated
RME-8 DnaJC13 23317 O75165 Functions in endosomal trafficking; human homolog of Receptor-mediated endocytosis 8 (RME8) in C. elegans; widely expressed, binds Hsc70
Tid1 hTid-1, DnaJA3 9093 Q96EY1 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; 10576 P78371 Component of hetero-oligomeric TRiC complex
CCT3 chaperonin containing TCP1 subunit 3 (gamma), CCTG, CCT-gamma, TRIC5, TCP1-gamma 7203 P49368 Component of hetero-oligomeric TRiC complex; three alternative splice forms
CCT4 chaperonin containing TCP1 subunit 4 (delta), SRB, CCTd 10575 P50991 Component of hetero-oligomeric TRiC complex
CCT5 chaperonin containing TCP1 subunit 5 (epsilon), CCTE, CCT-epsilon, TCP1-epsilon 22948 P48643 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 908 P40227 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 10693 Q92526 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 10574 Q99832 Component of hetero-oligomeric TRiC complex; two alternative splice forms
Hsp10 CPN10, GroES, HSPE1 3336 P61604 Closely linked to the Hsp60 gene (HspD1); forms chaperonin 'cap' structure
Hsp60 HspD1, CPN60, GroEL, HSP65, SPG13, HuCHA60 3329 P10809 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 6950 P17987 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 7184 P14625 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
retention in the ER
Hsp90 alpha Hsp90AA1, Hsp86, Hsp89 3320 P07900 Cytosolic form, induced by elevated temperature; ATPase activity;  three splice variants
Hsp90 alpha Hsp90AA2 3324 Q14568 Cytosolic form, induced by elevated temperature
Hsp90 beta Hsp90AB1 3326 P08238 Cytosolic form, constitutively expressed; ATPase activity; three splice variants
TRAP1 Hsp75 10131 Q12931 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 10598 O95433 Stimulates the inherent ATPase activity of Hsp90
Cdc37 NA 11140 Q16543 A chaperone that binds Hsp90 and is required for the activity of numerous protein kinases
Hop STIP1, STI1 10963 P31948 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 10728 Q15185 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 1409 P02489 Expression restricted to the lens
Crystallin, alpha B CRYAB, CRYA2, HSPB5 1410 P02511 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 26353 Q9UJY1 Charcot-Marie-Tooth disease type 2L; Hereditary motor neuropathy type II
heat shock 27kDa protein 1 Hsp27, Hsp25, HSPB1 3315 P04792 Charcot-Marie-Tooth disease, axonal, type 2F; distal hereditary motor neuropathy
heat shock 27kDa protein 2 HSPB2, MKBP 3316 Q16082 Associates with myotonic dystrophy protein kinase (DMPK)
heat shock 27kDa protein 3 HSPB3, HSPL27 8988 Q12988 Inhibitor of actiin polymerization
heat shock 27kDa protein family, member 7 (cardiovascular) cvHSP, HspB7 27129 Q9UBY9 Bovine βH-crystallin
heat shock protein, alpha-crystallin-related, B6 HSPB6, Hsp20 126393 O14558 Structural component of eye lens
heat shock protein, alpha-crystallin-related, B9 HSPB9, CT51 94086 Q9BQS6 Testis specific
outer dense fiber of sperm tails 1 HSPB10, ODFP, SODF, ODF1 4956 Q14990 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 1411 P05813 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 1412 P53672 Member of the acidic group of beta crystallins
Crystallin, beta A4 CRYBA4 1413 P53673 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 1414 P53674 Member of the basic group of beta crystallins
Crystallin, beta B2 CRYBB2 1415 P43320 Member of the basic group of beta crystallins; mutation found to cause type 2 cerulean cataracts
Crystallin, beta B3 CRYBB3 1417 P26998 Member of the basic group of beta crystallins
Crystallin, gamma A CRYGA, CRYG1, CRYG5 1418 P11844 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 1419 P07316 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 1420 P07315 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 1421 P07320 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 155051 Q496G6 beta-gamma hybrid crystallin; expressed in retina and lens nuclear fibers in rodents
Crystallin, gamma S CRYGS, CRYG8 1427 P22914 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 821 P27824 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 811 P27797 Calcium-binding ER protein; also found in the nucleus; can bind and inhibit nuclear hormone receptors
CHIP STUB1, UBOX1, HspABP2 10273 Q9UNE7 E3 ubiquitin ligase; co-chaperone that associates with Hsp70 and Hsp90
HO-1 heme oxygenase (decyclizing) 1, HMOX1, Hsp32 3162 P09601 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 3163 P30519 Non-inducible; cleaves heme ring at the alpha methene bridge to form biliverdin
HSF-1 HSTF1 3297 Q00613 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 3298 Q03933 Regulates the program of heat shock gene expression
Hsp47 SerpinH1, Colligin, Gp46 871 P50454 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 10945 P24390 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 11014 P33947 Two alternative splice forms
KDELR3 ERD2L3 11015 O43731 Two alternative splice forms
UGGT UDP-glucose:glycoprotein glucosyltransferase, HUGT1, UGCGL1, GT, UGT1, UGTR 56886 Q9NYU2 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 54431 Q8IXB1 Expression induced by ER stress; also a member of the DnaJ family
ERp18 TXNDC12, AGR1, ERp19 51060 O95881 Member of the thioredoxin superfamily; expressed in ER lumen
ERp27 NA 121506 Q96DN0 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 10961 P30040 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 23071 Q9BS26 Induced by ER stress
ERp46 TXNDC5 81567 Q8NBS9 Highly expressed in endothelial cells, where it is induced by and protects against hypoxia; contains 3 thioredoxin domains; two isoforms
ERp57 Grp58, PDIA3 2923 P30101 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 9601 P13667 Catalyzes rearrangement of disulfide bonds
P5 PDIA6, ERP5, TXNDC7 10130 Q15084 Can also function on the cell surface
PDI PDIA1, P4HB 5034 P07237 Catalyzes S-S bond formation, breakage, and rearrangement in nascent (ER lumen) and cell surface proteins
PDILT   204474 Q8IVQ5 Testis-specific expression
PDIP PDIA2 64714 Q13087 Catalyzes S-S bond rearrangement; expressed in ER lumen; high expression in pancreas
PDIR PDIA5 10954 Q14554 Contains 3 thioredoxin domains
TMX TXNDC1 81542 Q9H3N1 Contains one thioredoxin domain and a putative transmembrane domain
TMX2 TXNDC14 51075 Q561W0 (TrEMBL ID) Contains one thioredoxin domain and a putative transmembrane domain
TMX3 TXNDC10 54495 Q96JJ7 Contains one thioredoxin domain and a putative transmembrane domain
TMX4 TXNDC13 56255 Q9H1E5 Contains one thioredoxin domain and a putative transmembrane domain
 

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