Heat Shock Protein 47 (HSP47)

CBP2; AsTP3; CBP1; PIG14; RA-A47; SERPINH1; SERPINH2; Colligen; Gp46; Serpin Peptidase Inhibitor Clade H Member 1; Heat Shock Protein 47; Collagen Binding Protein 2

SPN	W493
HGNC	1546
MGI	88283
PubMed	9722680
RGD	69302
UniProt	P50454
Wiki	HSP47

Heat shock protein 47 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. Colligins are characterized by an amino acid structure that includes an N-terminal hydrophobic signal sequence and 2 putative N-linked oligosaccharide attachment sites (Clarke et al., 1991). Colligins also have a C-terminal RDEL sequence that acts as an endoplasmic reticulum (ER) retention sequence. Other features permit the colligin-binding protein of ER to be classified as a serpin (serine-arginine protease inhibitor).

Reference

"Cloning of a human collagen-binding protein, and its homology with rat gp46, chick hsp47 and mouse J6 proteins."
Biochim. Biophys. Acta 1129:246-248(1992) [PubMed] [Europe PMC] [Abstract]

"Isolation, characterization and chromosomal assignment of human colligin-2 gene (CBP2)."
Cytogenet. Cell Genet. 71:182-186(1995) [PubMed] [Europe PMC] [Abstract]

"Rheumatoid arthritis-related antigen 47kDa (RA-A47) is a product of colligin-2 and acts as a human HSP47."
J. Bone Miner. Metab. 18:328-334(2000) [PubMed] [Europe PMC] [Abstract]

"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]

"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"A functional SNP in the promoter of the SERPINH1 gene increases risk of preterm premature rupture of membranes in African Americans."
Proc. Natl. Acad. Sci. U.S.A. 103:13463-13467(2006) [PubMed] [Europe PMC] [Abstract]

"Initial characterization of the human central proteome."
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]

"Resveratrol-induced changes of the human adipocyte secretion profile."
J. Proteome Res. 11:4733-4743(2012) [PubMed] [Europe PMC] [Abstract]

"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]

"Homozygosity for a missense mutation in SERPINH1, which encodes the collagen chaperone protein HSP47, results in severe recessive osteogenesis imperfecta."
Am. J. Hum. Genet. 86:389-398(2010) [PubMed] [Europe PMC] [Abstract]