antibody

SERPINH1 antibody

MBS835022

0.1 mg

390 USD

polyclonal

rabbit

nonconjugated

human, mouse, rat, dog, chicken, cow, zebrafish

Antibody

SERPINH1 antibody

SERPINH1

Polyclonal SERPINH1; Anti-SERPINH1; SERPINH-1; heat shock protein 47; clade H; member 1; SERPINH 1; serpin peptidase inhibitor; SERPINH1; collagen binding

serpin H1; Serpin H1; serpin H1; 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

SERPINH1

SERPINH1; SERPINH1; CBP1; CBP2; OI10; gp46; AsTP3; HSP47; PIG14; PPROM; RA-A47; SERPINH2; CBP1; CBP2; HSP47; SERPINH2; AsTP3; Colligin

SERPH_HUMAN

Polyclonal

Rabbit

Human, Mouse, Rat, African Clawed Frog, Zebrafish, Bovine, Chicken, Dog

418

Lyophilized powder. Add 100 ul of distilled water. Final antibody concentration is 1 mg/ml in PBS buffer.

1 mg/ml

Store at 4 degree C, following reconstitution, aliquot and store at -20 degree C. Avoid repeated freeze-thaw cycles.

Western Blot (WB)

Optimal conditions to be determined by the end user

Biological Significance: Serine (or cysteine) proteinase inhibitor, clade H (heat shock protein 47), member 1, (collagen binding protein 1)

Immunogen: SERPINH1 antibody was raised in rabbit using the C terminal of SERPINH1 as the immunogen

Signal Transduction

Rabbit polyclonal SERPINH1 antibody

32454741

NP_001226

NM_001235.3

P50454

46,441 Da

Collagen Biosynthesis And Modifying Enzymes Pathway (645289); Collagen Formation Pathway (645288); Endochondral Ossification Pathway (198812); Extracellular Matrix Organization Pathway (576262)

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]

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

0.1 mg

390 USD