SERPINH1
Description
The SERPINH1 (serpin family H member 1) is a protein-coding gene located on chromosome 11.
Heat shock protein 47, also known as SERPINH1, is a serpin which serves as a human chaperone protein for collagen. It is a member of the serpin superfamily of serine proteinase inhibitors. Its expression is induced by heat shock. HSP47 is expressed in the endoplasmic reticulum, where cells synthesize and secrete type I and type II collagen. 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. HSP47 is essential for the correct folding of procollagen. Antibodies directed to this protein have been found in patients with rheumatoid arthritis. HSP47 contains 3 beta sheets and 9 alpha helices. After binding with collagen no conformation change is observed.
SERPINH1 is also known as AsTP3, CBP1, CBP2, HSP47, OI10, PIG14, PPROM, RA-A47, SERPINH2, gp46.
