Serpin H1 (SERPINH1) Antibody

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Description
Serpin H1 (SERPINH1) Antibody is a Recombinant Rabbit Monoclonal antibody for the detection of Human SERPINH1.
Documents del producto
Product specifications
Category | Primary Antibodies |
Immunogen Target | Serpin H1 (SERPINH1) |
Host | Rabbit |
Reactivity | Human |
Recommended Dilution | IHC: 1/50 - 1/200. Optimal dilutions/concentrations should be determined by the end user. |
Clonality | Monoclonal |
Conjugation | Unconjugated |
Isotype | IgG |
Expression | Recombinant |
Purification | Purified by affinity chromatography. |
Size 1 | 50 µl |
Size 2 | 100 µl |
Form | Liquid |
Tested Applications | ELISA, IHC |
Buffer | PBS, pH 7.4, 150 mM NaCl, 0.02% sodium azide and 50% glycerol. |
Availability | Shipped within 5-10 working days. |
Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
Dry Ice | No |
UniProt ID | P50454 |
Alias | CBP1,CBP2,OI10,gp46,AsTP3,HSP47,PIG14,PPROM,RA-A47,SERPINH2,Serpin H1,Arsenic-transactivated protein 3,Collagen-binding protein,Colligin,Rheumatoid arthritis-related antigen,RA-A47,47 kDa heat shock protein |
Background | Antibody anti-SERPINH1 |
Status | RUO |
Descripción
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.
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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.
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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.
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