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Gene Review

SERPINH1  -  serpin peptidase inhibitor, clade H (heat...

Homo sapiens

Synonyms: 47 kDa heat shock protein, Arsenic-transactivated protein 3, AsTP3, CBP1, CBP2, ...
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Disease relevance of SERPINH1


High impact information on SERPINH1

  • Saccharomyces cerevisiae mutants lacking the CBP1 homolog RCN1 are, like calcineurin mutants, sensitive to lithium cation stress [7].
  • The 4-hydroxyproline-containing procollagen polypeptide chains were of full length and formed molecules with stable triple helices even though Pichia probably has no Hsp47-like protein [8].
  • Anchorage-dependent cell lines like L6 myoblasts and normal rat kidney fibroblasts produced both colligin and procollagen I mRNA, but the level of both was concurrently reduced considerably in their ras-transformed counterparts [9].
  • Similarly, during the differentiation of L6 myoblasts, levels of both colligin and procollagen declined together [9].
  • Parallel regulation of procollagen I and colligin, a collagen-binding protein and a member of the serine protease inhibitor family [9].

Chemical compound and disease context of SERPINH1


Biological context of SERPINH1


Anatomical context of SERPINH1

  • Boyden chamber tumor invasion assays and colloidal gold migration assays utilizing a reconstituted basement membrane (Matrigel), collagen type I, and laminin-5 substrates revealed that cell lines expressing constitutive high levels of colligin/Hsp47 manifested the lowest invasion and migration indices [3].
  • These data indicate that colligin/Hsp47 is anchored to the cell membrane in a complex with CD9 where it moderates tumor cell invasion and motility possibly by acting as a serpin protein inhibitor or as a receptor for collagen [3].
  • Together, these findings indicate a novel means by which type I collagen production is regulated by the endoplasmic reticulum constituent, Hsp47, and suggest a potential basis for inherent differences in collagen production within the population [5].
  • To evaluate the effects of Hsp47 on collagen production we infected human vascular smooth muscle cells (SMCs) with a retrovirus containing Hsp47 cDNA [5].
  • To show that the isolated cDNA encodes RA-A47 protein, it was expressed in Cos-7 cells [17].

Associations of SERPINH1 with chemical compounds


Physical interactions of SERPINH1

  • Both PDI and the collagen-binding protein hsp47 showed a similar pH-dependent interaction with folded collagen, dissociating when the pH was lowered to pH 6 [21].

Regulatory relationships of SERPINH1


Other interactions of SERPINH1


Analytical, diagnostic and therapeutic context of SERPINH1

  • Compared to the controls, an increased expression of type I collagen, type III collagen and HSP47 was detected in conjunctival biopsy sections of patient with EBA using immunohistochemistry [4].
  • In animal models of liver injury, hepatic stellate cells (HSC) have been identified as a source of HSP47 [10].
  • When dermal fibroblasts were treated with various concentrations of TGF-beta 1 (6.25, 12.5, 25, 50 and 100 ng/ml for 24 h), it induced the expression of both type I collagen and HSP47, as determined by quantitative real-time PCR [26].
  • The goal of the present study was to examine the possible involvement of two important wound-healing-associated proteins-heat shock protein 47 (Hsp47), fibronectin (Fn), and its oncofetal isoforms-in DD, using clinical tissue samples and primary cell cultures [27].
  • The level of HSP 47 and/or procollagen expression in fibroblasts was measured by real-time RT-PCR (mRNA transcripts) and by flow cytometry (immunochemistry technique for measurement of arbitrary fluorescence intensity) [28].


  1. Downregulation of rheumatoid arthritis-related antigen RA-A47 (HSP47/colligin-2) in chondrocytic cell lines induces apoptosis and cell-surface expression of RA-A47 in association with CD9. Hattori, T., von der Mark, K., Kawaki, H., Yutani, Y., Kubota, S., Nakanishi, T., Eberspaecher, H., de Crombrugghe, B., Takigawa, M. J. Cell. Physiol. (2005) [Pubmed]
  2. Molecular epidemiology of simian T-lymphotropic virus (STLV) in wild-caught monkeys and apes from Cameroon: a new STLV-1, related to human T-lymphotropic virus subtype F, in a Cercocebus agilis. Nerrienet, E., Meertens, L., Kfutwah, A., Foupouapouognigni, Y., Gessain, A. J. Gen. Virol. (2001) [Pubmed]
  3. Cell surface colligin/Hsp47 associates with tetraspanin protein CD9 in epidermoid carcinoma cell lines. Hebert, C., Norris, K., Della Coletta, R., Reynolds, M., Ordóñez, J., Sauk, J.J. J. Cell. Biochem. (1999) [Pubmed]
  4. Expression profiles of collagens, HSP47, TGF-beta1, MMPs and TIMPs in epidermolysis bullosa acquisita. Razzaque, M.S., Kumari, S., Foster, C.S., Ahmed, A.R. Cytokine (2003) [Pubmed]
  5. Functional linkage between the endoplasmic reticulum protein Hsp47 and procollagen expression in human vascular smooth muscle cells. Rocnik, E.F., van der Veer, E., Cao, H., Hegele, R.A., Pickering, J.G. J. Biol. Chem. (2002) [Pubmed]
  6. A functional proteomic method for the enrichment of peripheral membrane proteins reveals the collagen binding protein Hsp47 is exposed on the surface of activated human platelets. Kaiser, W.J., Holbrook, L.M., Tucker, K.L., Stanley, R.G., Gibbins, J.M. J. Proteome Res. (2009) [Pubmed]
  7. Identification and characterization of a highly conserved calcineurin binding protein, CBP1/calcipressin, in Cryptococcus neoformans. Görlach, J., Fox, D.S., Cutler, N.S., Cox, G.M., Perfect, J.R., Heitman, J. EMBO J. (2000) [Pubmed]
  8. Assembly of human prolyl 4-hydroxylase and type III collagen in the yeast pichia pastoris: formation of a stable enzyme tetramer requires coexpression with collagen and assembly of a stable collagen requires coexpression with prolyl 4-hydroxylase. Vuorela, A., Myllyharju, J., Nissi, R., Pihlajaniemi, T., Kivirikko, K.I. EMBO J. (1997) [Pubmed]
  9. Parallel regulation of procollagen I and colligin, a collagen-binding protein and a member of the serine protease inhibitor family. Clarke, E.P., Jain, N., Brickenden, A., Lorimer, I.A., Sanwal, B.D. J. Cell Biol. (1993) [Pubmed]
  10. Expression of HSP47, a collagen-specific chaperone, in normal and diseased human liver. Brown, K.E., Broadhurst, K.A., Mathahs, M.M., Brunt, E.M., Schmidt, W.N. Lab. Invest. (2005) [Pubmed]
  11. Antibody reactivity to different regions of human T-cell leukemia virus type 1 gp61 in infected people. Chen, Y.M., Lee, T.H., Samuel, K.P., Okayama, A., Tachibana, N., Miyoshi, I., Papas, T.S., Essex, M. J. Virol. (1989) [Pubmed]
  12. Antisense oligonucleotides against collagen-binding stress protein HSP47 suppress peritoneal fibrosis in rats. Nishino, T., Miyazaki, M., Abe, K., Furusu, A., Mishima, Y., Harada, T., Ozono, Y., Koji, T., Kohno, S. Kidney Int. (2003) [Pubmed]
  13. Immunolocalization of collagen and collagen-binding heat shock protein 47 in fibrotic lung diseases. Razzaque, M.S., Nazneen, A., Taguchi, T. Mod. Pathol. (1998) [Pubmed]
  14. Partial characterization of a collagen-binding, differentiation-related glycoprotein from skeletal myoblasts. Nandan, D., Cates, G.A., Ball, E.H., Sanwal, B.D. Arch. Biochem. Biophys. (1990) [Pubmed]
  15. 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. 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. J. Immunol. (2002) [Pubmed]
  16. The human genome has only one functional hsp47 gene (CBP2) and a pseudogene (pshsp47). Nagai, N., Tetuya, Y., Hosokawa, N., Nagata, K. Gene (1999) [Pubmed]
  17. Rheumatoid arthritis-related antigen 47kDa (RA-A47) is a product of colligin-2 and acts as a human HSP47. Hattori, T., Takahash, K., Yutani, Y., Fujisawa, T., Nakanishi, T., Takigawa, M. J. Bone Miner. Metab. (2000) [Pubmed]
  18. Isolation, characterization and chromosomal assignment of human colligin-2 gene (CBP2). Ikegawa, S., Sudo, K., Okui, K., Nakamura, Y. Cytogenet. Cell Genet. (1995) [Pubmed]
  19. Exogenous nitric oxide enhances the synthesis of type I collagen and heat shock protein 47 by normal human dermal fibroblasts. Obayashi, K., Akamatsu, H., Okano, Y., Matsunaga, K., Masaki, H. J. Dermatol. Sci. (2006) [Pubmed]
  20. The production of the endostatin precursor collagen XVIII in head and neck carcinomas is modulated by CBP2/Hsp47. Ollins, G.J., Nikitakis, N., Norris, K., Herbert, C., Siavash, H., Sauk, J.J. Anticancer Res. (2002) [Pubmed]
  21. Thiol-independent interaction of protein disulphide isomerase with type X collagen during intra-cellular folding and assembly. McLaughlin, S.H., Bulleid, N.J. Biochem. J. (1998) [Pubmed]
  22. Role of collagen-binding heat shock protein 47 and transforming growth factor-beta1 in conjunctival scarring in ocular cicatricial pemphigoid. Razzaque, M.S., Foster, C.S., Ahmed, A.R. Invest. Ophthalmol. Vis. Sci. (2003) [Pubmed]
  23. Heat shock protein 47 is expressed in fibrous regions of human atheroma and Is regulated by growth factors and oxidized low-density lipoprotein. Rocnik, E., Chow, L.H., Pickering, J.G. Circulation (2000) [Pubmed]
  24. Isolation and characterization of a rheumatoid arthritis-specific antigen (RA-A47) from a human chondrocytic cell line (HCS-2/8). Hattori, T., Fujisawa, T., Sasaki, K., Yutani, Y., Nakanishi, T., Takahashi, K., Takigawa, M. Biochem. Biophys. Res. Commun. (1998) [Pubmed]
  25. Structure of the gene encoding human colligin-2 (CBP2). Ikegawa, S., Nakamura, Y. Gene (1997) [Pubmed]
  26. Collagens, collagen-binding heat shock protein 47 and transforming growth factor-beta 1 are induced in cicatricial pemphigoid: possible role(s) in dermal fibrosis. Razzaque, M.S., Ahmed, A.R. Cytokine (2002) [Pubmed]
  27. Wound healing-associated proteins Hsp47 and fibronectin are elevated in Dupuytren's contracture. Howard, J.C., Varallo, V.M., Ross, D.C., Faber, K.J., Roth, J.H., Seney, S., Gan, B.S. J. Surg. Res. (2004) [Pubmed]
  28. Heat shock protein 47 expression in aged normal human fibroblasts: modulation by Salix alba extract. Nizard, C., Noblesse, E., Boisdé, C., Moreau, M., Faussat, A.M., Schnebert, S., Mahé, C. Ann. N. Y. Acad. Sci. (2004) [Pubmed]
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