Serpin H1 (Serpinh1) Antibody (FITC)

Background

SERPINH1, also known as heat shock protein 47 (HSP47), is a member of the serpin (serine protease inhibitor) superfamily, though it functions differently from classical serpins. Unlike other serpins, SERPINH1 is not a protease inhibitor but rather acts as a molecular chaperone. It plays a crucial role in collagen biosynthesis, aiding the proper folding and stabilization of collagen molecules during their formation. As a collagen-specific chaperone, it is essential in maintaining the structural integrity of connective tissues and contributes significantly to tissue development and repair. The SERPINH1 gene is located on chromosome 11 in humans. Mutations in this gene have been associated with connective tissue disorders, notably osteogenesis imperfecta (OI), a condition characterized by fragile bones.