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Anti-Hsp47/SERPINH1 Antibody Picoband® Fluoro647 Conjugated

Product Specifications

Background

Heat shock protein 47, also known as SERPINH1 or HSP47, is a serpin which serves as a human chaperone protein for collagen. This protein 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. It has been found that HSP47 monitors the integrity of the triple helix of type I procollagen at the ER/cis-Golgi boundary and, when absent, the rate of transit from the ER to the Golgi is increased and the helical structure is compromised.

Synonyms

Serpin H1;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; CBP1, CBP2, HSP47, SERPINH2; PIG14

Gene Name

SERPINH1

Gene ID

871

UniProt

P50454

Host

Rabbit

Reactivity

Human, Mouse, Rat

Cross Reactivity

No cross-reactivity with other proteins

Immunogen

E.coli-derived human Hsp47 recombinant protein (Position: D247-L418) . Human Hsp47 shares 97% amino acid (aa) sequence identity with both mouse and rat Hsp47.

Clonality

Polyclonal

Tissue Specificity

Highly expressed in testis and thymus, followed by small intestine, placenta, colon, pancreas and ovary. Weakly expressed in breast.

Applications

Flow Cytometry

Field of Research

Chaperones, Developmental Biology, Heat Shock Proteins, Organogenesis, Protein Trafficking, Signal Transduction, Skeletal Development

Purification

Immunogen affinity purified.

Form

Liquid

Function

Binds specifically to collagen. Could be involved as a chaperone in the biosynthetic pathway of collagen.

References & Citations

1. Christiansen, H. E., Schwarze, U., Pyott, S. M., AlSwaid, A., Al Balwi, M., Alrasheed, S., Pepin, M. G., Weis, M. A., Eyre, D. R., Byers, P. H. Homozygosity for a missense mutation in SERPINH1, which encodes the collagen chaperone protein HSP47, results in severe recessive osteogenesis imperfecta. Am. J. Hum. Genet. 86: 389-398, 2010. 2. Entrez Gene: SERPINH1 serpin peptidase inhibitor, clade H (heat shock protein 47), member 1, (collagen binding protein 1) 3. Dafforn T, Della M, Miller A (2001) . The molecular interactions of heat shock protein 47 (Hsp47) and their implications for collagen biosynthesis. J. Biol. Chem. 276 (52) : 49310–9.

Storage Conditions

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Calculated Molecular Weight

46441 MW

Specificity

No cross reactivity with other proteins.

Applications Notes

6

Gene Name Synonym

Serpin H1

Subcellular Location

Endoplasmic reticulum lumen.

Sequence Similarities

Belongs to the serpin family.

Protein Name

Serpin H1

Isotype

Rabbit IgG

Contents

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.

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