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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

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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