Heat shock protein 47

Serpin peptidase inhibitor, clade H (heat shock protein 47), member 1, (collagen binding protein 1)
Identifiers
Symbols	SERPINH1 ; AsTP3; CBP1; CBP2; HSP47; OI10; PIG14; PPROM; RA-A47; SERPINH2; gp46
External IDs	OMIM: 600943 MGI: 88283 HomoloGene: 20331 ChEMBL: 5286 GeneCards: SERPINH1 Gene
Gene ontology Molecular function • serine-type endopeptidase inhibitor activity • collagen binding • poly(A) RNA binding • unfolded protein binding Cellular component • extracellular space • endoplasmic reticulum • endoplasmic reticulum lumen • endoplasmic reticulum-Golgi intermediate compartment • membrane raft • extracellular exosome Biological process • chondrocyte development involved in endochondral bone morphogenesis • response to unfolded protein • negative regulation of endopeptidase activity • extracellular matrix organization • collagen fibril organization • collagen biosynthetic process • protein maturation Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	871	12406
Ensembl	ENSG00000149257	ENSMUSG00000070436
UniProt	P50454	P19324
RefSeq (mRNA)	NM_001207014	NM_001111043
RefSeq (protein)	NP_001193943	NP_001104513
Location (UCSC)	Chr 11: 75.56 – 75.57 Mb	Chr 7: 99.35 – 99.35 Mb
PubMed search

Heat shock protein 47, also known as SERPINH1 is a serpin which serves as a human chaperone protein for collagen.^[1][2]

Function

This protein is a member of the serpin superfamily of serine proteinase inhibitors. Its expression is induced by heat shock. The protein localizes to the endoplasmic reticulum lumen and binds collagen; thus it is thought to be a molecular chaperone involved in the maturation of collagen molecules. Autoantibodies to this protein have been found in patients with rheumatoid arthritis.^[1]

Interactions

Heat shock protein 47 has been shown to interact with collagens I, II, III, IV and V.^[3]

References

1. "Entrez Gene: SERPINH1 serpin peptidase inhibitor, clade H (heat shock protein 47), member 1, (collagen binding protein 1)".
2. Dafforn TR, Della M, Miller AD (2001). "The molecular interactions of heat shock protein 47 (Hsp47) and their implications for collagen biosynthesis". J. Biol. Chem. 276 (52): 49310–9. doi:10.1074/jbc.M108896200. PMID 11592970.
3. Mala JG, Rose C (2010). "Interactions of heat shock protein 47 with collagen and the stress response: An unconventional chaperone model?". Life Sciences 87 (19-22): 579–86. doi:10.1016/j.lfs.2010.09.024. PMID 20888348.

Further reading

• Razzaque MS, Taguchi T (1999). "The possible role of colligin/HSP47, a collagen-binding protein, in the pathogenesis of human and experimental fibrotic diseases.". Histol. Histopathol. 14 (4): 1199–212. PMID 10506936.
• Gettins PG (2003). "Serpin structure, mechanism, and function.". Chem. Rev. 102 (12): 4751–804. doi:10.1021/cr010170. PMID 12475206.
• Clarke EP, Sanwal BD (1992). "Cloning of a human collagen-binding protein, and its homology with rat gp46, chick hsp47 and mouse J6 proteins.". Biochim. Biophys. Acta 1129 (2): 246–8. doi:10.1016/0167-4781(92)90498-o. PMID 1309665.
• Ikegawa S, Sudo K, Okui K, Nakamura Y (1995). "Isolation, characterization and chromosomal assignment of human colligin-2 gene (CBP2).". Cytogenet. Cell Genet. 71 (2): 182–6. doi:10.1159/000134103. PMID 7656593.
• Ikegawa S, Nakamura Y (1997). "Structure of the gene encoding human colligin-2 (CBP2).". Gene 194 (2): 301–3. doi:10.1016/S0378-1119(97)00209-6. PMID 9272875.
• Bekri S, Adélaïde J, Merscher S, Grosgeorge J, Caroli-Bosc F, Perucca-Lostanlen D, Kelley PM, Pébusque MJ, Theillet C, Birnbaum D, Gaudray P (1998). "Detailed map of a region commonly amplified at 11q13→q14 in human breast carcinoma.". Cytogenet. Cell Genet. 79 (1–2): 125–31. doi:10.1159/000134699. PMID 9533029.
• Hattori T, Fujisawa T, Sasaki K, Yutani Y, Nakanishi T, Takahashi K, Takigawa M (1998). "Isolation and characterization of a rheumatoid arthritis-specific antigen (RA-A47) from a human chondrocytic cell line (HCS-2/8)". Biochem. Biophys. Res. Commun. 245 (3): 679–83. doi:10.1006/bbrc.1998.8505. PMID 9588174.
• Nagai N, Tetuya Y, Hosokawa N, Nagata K (1999). "The human genome has only one functional hsp47 gene (CBP2) and a pseudogene (pshsp47)". Gene 227 (2): 241–8. doi:10.1016/S0378-1119(98)00592-7. PMID 10023073.
• Hebert C, Norris K, Della Coletta R, Reynolds M, Ordóñez J, Sauk JJ (1999). "Cell surface colligin/Hsp47 associates with tetraspanin protein CD9 in epidermoid carcinoma cell lines". J. Cell. Biochem. 73 (2): 248–58. doi:10.1002/(SICI)1097-4644(19990501)73:2<248::AID-JCB11>3.0.CO;2-A. PMID 10227388.
• Koide T, Aso A, Yorihuzi T, Nagata K (2000). "Conformational requirements of collagenous peptides for recognition by the chaperone protein HSP47". J. Biol. Chem. 275 (36): 27957–63. doi:10.1074/jbc.M003026200. PMID 10862616.
• Hattori T, Takahash K, Yutani Y, Fujisawa T, Nakanishi T, Takigawa M (2001). "Rheumatoid arthritis-related antigen 47kDa (RA-A47) is a product of colligin-2 and acts as a human HSP47". J. Bone Miner. Metab. 18 (6): 328–34. doi:10.1007/s007740070004. PMID 11052465.
• Pelletier G, Stefanovsky VY, Faubladier M, Hirschler-Laszkiewicz I, Savard J, Rothblum LI, Côté J, Moss T (2002). "Competitive recruitment of CBP and Rb-HDAC regulates UBF acetylation and ribosomal transcription". Mol. Cell 6 (5): 1059–66. doi:10.1016/S1097-2765(00)00104-0. PMID 11106745.
• Matsuda M, Koide T, Yorihuzi T, Hosokawa N, Nagata K (2001). "Molecular cloning of a novel ubiquitin-like protein, UBIN, that binds to ER targeting signal sequences". Biochem. Biophys. Res. Commun. 280 (2): 535–40. doi:10.1006/bbrc.2000.4149. PMID 11162551.
• Zhou M, Nekhai S, Bharucha DC, Kumar A, Ge H, Price DH, Egly JM, Brady JN (2002). "TFIIH inhibits CDK9 phosphorylation during human immunodeficiency virus type 1 transcription". J. Biol. Chem. 276 (48): 44633–40. doi:10.1074/jbc.M107466200. PMID 11572868.
• Song CZ, Keller K, Murata K, Asano H, Stamatoyannopoulos G (2002). "Functional interaction between coactivators CBP/p300, PCAF, and transcription factor FKLF2". J. Biol. Chem. 277 (9): 7029–36. doi:10.1074/jbc.M108826200. PMC 2808425. PMID 11748222.
• Sasaki H, Sato T, Yamauchi N, Okamoto T, Kobayashi D, Iyama S, Kato J, Matsunaga T, Takimoto R, Takayama T, Kogawa K, Watanabe N, Niitsu Y (2002). "Induction of heat shock protein 47 synthesis by TGF-beta and IL-1 beta via enhancement of the heat shock element binding activity of heat shock transcription factor 1". J. Immunol. 168 (10): 5178–83. doi:10.4049/jimmunol.168.10.5178. PMID 11994473.
• Razzaque MS, Ahmed AR (2002). "Collagens, collagen-binding heat shock protein 47 and transforming growth factor-beta 1 are induced in cicatricial pemphigoid: possible role(s) in dermal fibrosis". Cytokine 17 (6): 311–6. doi:10.1006/cyto.2002.1020. PMID 12061838.
• Sato K, Yomogida K, Wada T, Yorihuzi T, Nishimune Y, Hosokawa N, Nagata K (2002). "Type XXVI collagen, a new member of the collagen family, is specifically expressed in the testis and ovary". J. Biol. Chem. 277 (40): 37678–84. doi:10.1074/jbc.M205347200. PMID 12145293.
• Rocnik EF, van der Veer E, Cao H, Hegele RA, Pickering JG (2002). "Functional linkage between the endoplasmic reticulum protein Hsp47 and procollagen expression in human vascular smooth muscle cells". J. Biol. Chem. 277 (41): 38571–8. doi:10.1074/jbc.M206689200. PMID 12163502.

External links

• The MEROPS online database for peptidases and their inhibitors: I04.035
• HSP47 Heat-Shock Proteins at the US National Library of Medicine Medical Subject Headings (MeSH)