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Mouse Anti-SERPINH1 Recombinant Antibody (CBFYH-2754) (CBMAB-H3817-FY)


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Summary

Host Animal
Mouse
Specificity
Mouse, Rat, Human
Clone
CBFYH-2754
Application
WB, IP, IF, ELISA

Basic Information

Specificity
Mouse, Rat, Human
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
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Serpin Family H Member 1
Introduction
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.
Entrez Gene ID
Human871
Mouse12406
Rat29345
UniProt ID
HumanP50454
MouseP19324
RatP29457
Alternative Names
Serpin Family H Member 1; Serine (Or Cysteine) Proteinase Inhibitor, Clade H (Heat Shock Protein 47), Member 1, (Collagen Binding Protein 1); Serpin Peptidase Inhibitor, Clade H (Heat Shock Protein 47), Member 1, (Collagen Binding Protein 1); Cell Proliferation-Inducing Gene 14 Protein; Rheumatoid Arthritis-Related Antigen RA-A47; Arsenic-Transactivated Protein 3; Collagen Binding Protein 1; 47 KDa Heat Shock Protein; SERPINH2; AsTP3; HSP47; CBP1; CBP2; Serine (Or Cysteine) Proteinase Inhibitor, Clade H (Heat Shock Protein 47), Member 2, (Collagen-Binding Protein 2)
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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