NP_001226
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
NCBI Official Full Name
serpin H1
NCBI Official Synonym Full Names
serpin peptidase inhibitor, clade H (heat shock protein 47), member 1, (collagen binding protein 1)
NCBI Official Synonym Symbols
CBP1; CBP2; OI10; gp46; AsTP3; HSP47; PIG14; PPROM; RA-A47; SERPINH2 [Similar Products]
NCBI Protein Information
serpin H1
UniProt Protein Name
Serpin H1
UniProt Synonym Protein Names
47 kDa heat shock protein; Arsenic-transactivated protein 3; AsTP3; Cell proliferation-inducing gene 14 protein; Collagen-binding protein; Colligin; Rheumatoid arthritis-related antigen RA-A47
UniProt Synonym Gene Names
UniProt Entry Name
SERPH_HUMAN
NCBI Summary for SERPINH1
This gene encodes a member of the serpin superfamily of serine proteinase inhibitors. The encoded protein is localized to the endoplasmic reticulum and plays a role in collagen biosynthesis as a collagen-specific molecular chaperone. Autoantibodies to the encoded protein have been found in patients with rheumatoid arthritis. Expression of this gene may be a marker for cancer, and nucleotide polymorphisms in this gene may be associated with preterm birth caused by preterm premature rupture of membranes. Alternatively spliced transcript variants have been observed for this gene, and a pseudogene of this gene is located on the short arm of chromosome 9. [provided by RefSeq, May 2011]
UniProt Comments for SERPINH1
SERPINH1: Binds specifically to collagen. Could be involved as a chaperone in the biosynthetic pathway of collagen. Defects in SERPINH1 are the cause of osteogenesis imperfecta type 10 (OI10). A connective tissue disorder characterized by bone fragility, low bone mass, bowing of limbs due to multiple fractures, short limb dwarfism and blue sclerae. Belongs to the serpin family.
Chromosomal Location of Human Ortholog: 11q13.5
Cellular Component: extracellular space; endoplasmic reticulum lumen; endoplasmic reticulum; ER-Golgi intermediate compartment
Molecular Function: serine-type endopeptidase inhibitor activity; collagen binding; unfolded protein binding
Biological Process: extracellular matrix organization and biogenesis; collagen fibril organization; protein maturation; collagen biosynthetic process; response to unfolded protein
Disease: Osteogenesis Imperfecta, Type X; Preterm Premature Rupture Of The Membranes
Research Articles on SERPINH1
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Products associated with anti-SERPINH1 antibody
Pathways associated with anti-SERPINH1 antibody
Diseases associated with anti-SERPINH1 antibody
Organs/Tissues associated with anti-SERPINH1 antibody
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