catalog number :
MBS6002005
products full name :
SERPINH1, ID (Serine/Cysteine Proteinase Inhibitor, Clade H, Member 1, CBP1, CBP2, RA-A47, gp46)r
products short name :
[SERPINH1]
products name syn :
[Anti -SERPINH1, ID (Serine/Cysteine Proteinase Inhibitor, Clade H, Member 1, CBP1, CBP2, RA-A47, gp46)r]
other names :
[serpin H1; Serpin H1; serpin H1; colligin-1; colligin-2; 47 kDa heat shock protein; arsenic-transactivated protein 3; rheumatoid arthritis antigen A-47; cell proliferation-inducing gene 14 protein; rheumatoid arthritis-related antigen RA-A47; serine (or cysteine) proteinase inhibitor, clade H (heat shock protein 47), member 1, (collagen binding protein 1); serine (or cysteine) proteinase inhibitor, clade H (heat shock protein 47), member 2, (collagen-binding protein 2); serpin peptidase inhibitor, clade H (heat shock protein 47), member 1, (collagen binding protein 1); 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]
other gene names :
[SERPINH1; SERPINH1; CBP1; CBP2; OI10; gp46; AsTP3; HSP47; PIG14; PPROM; RA-A47; SERPINH2; CBP1; CBP2; HSP47; SERPINH2; AsTP3; Colligin]
uniprot entry name :
SERPH_HUMAN
specificity :
Recognizes rat SERPINH1. Species Crossreactivity: human
purity :
Affinity Purified. Purified by Protein G affinity chromatography.
form :
Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.
concentration :
0.38 mg/mL
storage stability :
May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
tested application :
ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC). Other applications have not been tested.
app notes :
Western Blot: 1:100-1:200. Immunohistochemistry (FFPE): 1:50-1:100 . Optimal dilutions to be determined by the researcher.
image1 heading :
Western Blot (WB)
image2 heading :
Immunohistochemistry (IHC)
other info1 :
Immunogen: KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 222-251 in the Central region of rat SERPINH1. Species sequence homolgy: bovine and mouse.
products categories :
Antibodies; Abs to Protease Inhibitors
products description :
SERPINH1 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.
ncbi acc num :
NP_001193943.1
ncbi gb acc num :
NM_001207014.1
ncbi pathways :
Collagen Biosynthesis And Modifying Enzymes Pathway (645289); Collagen Formation Pathway (645288); Endochondral Ossification Pathway (198812); Extracellular Matrix Organization Pathway (576262)
ncbi summary :
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 summary :
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