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Mouse Anti-SERPINH1 Recombinant Antibody (1D2-1A6) (CBMAB-A8007-LY)


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Summary

Host Animal
Mouse
Specificity
Human, Rat
Clone
1D2-1A6
Antibody Isotype
IgG1, κ
Application
WB, ELISA

Basic Information

Immunogen
SERPINH1 (AAH14623.1, 1 a.a. ~ 418 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Specificity
Human, Rat
Antibody Isotype
IgG1, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Serpin Family H Member 1
Introduction
This gene encodes 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. [provided by RefSeq]
Entrez Gene ID
Human871
Rat29345
UniProt ID
HumanP50454
RatP29457
Alternative Names
AsTP3; CBP1; CBP2; HSP47; PIG14; PPROM; RA-A47; SERPINH2; gp46
Function
Binds specifically to collagen. Could be involved as a chaperone in the biosynthetic pathway of collagen.
Biological Process
Biological Process chondrocyte development involved in endochondral bone morphogenesisIEA:Ensembl
Biological Process collagen biosynthetic processIEA:Ensembl
Biological Process collagen fibril organizationManual Assertion Based On ExperimentIBA:GO_Central
Biological Process negative regulation of endopeptidase activityManual Assertion Based On ExperimentIBA:GO_Central
Biological Process protein maturationIEA:Ensembl
Biological Process response to unfolded proteinManual Assertion Based On ExperimentTAS:ProtInc
Cellular Location
Endoplasmic reticulum lumen
Involvement in disease
Endoplasmic reticulum lumen:
A form of osteogenesis imperfecta, a connective tissue disorder characterized by low bone mass, bone fragility and susceptibility to fractures after minimal trauma. Disease severity ranges from very mild forms without fractures to intrauterine fractures and perinatal lethality. Extraskeletal manifestations, which affect a variable number of patients, are dentinogenesis imperfecta, hearing loss, and blue sclerae. OI10 is an autosomal recessive form characterized by multiple bone deformities and fractures, generalized osteopenia, dentinogenesis imperfecta, and blue sclerae.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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