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Mouse Anti-HJV Recombinant Antibody (1D7) (CBMAB-R0542-CN)

This product is a Mouse antibody that recognizes HJV. The antibody 1D7 can be used for immunoassay techniques such as: ELISA, FC, IP, WB.
See all HJV antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
1D7
Antibody Isotype
IgG1
Application
ELISA, FC, IP, WB

Basic Information

Immunogen
Recombinant human RGMC (aa. 36-398) (Prod. No. ALX-522-104)
Specificity
Human
Antibody Isotype
IgG1
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
Buffer
PBS
Preservative
0.02% Sodium azide

Target

Full Name
hemojuvelin BMP co-receptor
Introduction
The product of this gene is involved in iron metabolism. It may be a component of the signaling pathway which activates hepcidin or it may act as a modulator of hepcidin expression. It could also represent the cellular receptor for hepcidin. Defects in this gene are the cause of hemochromatosis type 2A, also called juvenile hemochromatosis (JH). JH is an early-onset autosomal recessive disorder due to severe iron overload resulting in hypogonadotrophic hypogonadism, hepatic fibrosis or cirrhosis and cardiomyopathy, occurring typically before age of 30. [provided by RefSeq, Oct 2015]
Entrez Gene ID
148738
UniProt ID
Q6ZVN8
Alternative Names
JH; HFE2; RGMC; HFE2A
Function
Acts as a bone morphogenetic protein (BMP) coreceptor (PubMed:18976966).

Through enhancement of BMP signaling regulates hepcidin (HAMP) expression and regulates iron homeostasis (PubMed:18976966).
Biological Process
Activin receptor signaling pathway Source: BHF-UCL
BMP signaling pathway Source: BHF-UCL
Cellular iron ion homeostasis Source: BHF-UCL
Cellular response to BMP stimulus Source: BHF-UCL
Iron ion homeostasis Source: MGI
Negative regulation of BMP signaling pathway Source: Ensembl
Negative regulation of transcription by RNA polymerase II Source: BHF-UCL
Positive regulation of transcription by RNA polymerase II Source: BHF-UCL
Protein autoprocessing Source: BHF-UCL
Cellular Location
Cell membrane. Also released in the extracellular space.
Involvement in disease
Hemochromatosis 2A (HFE2A):
A juvenile form of hemochromatosis, a disorder of iron metabolism with excess deposition of iron in a variety of organs leading to their failure, bronze skin pigmentation, hepatic cirrhosis, arthropathy and diabetes. The most common symptoms of juvenile hemochromatosis at presentation are hypogonadism and cardiomyopathy.
PTM
Autocatalytically cleaved at low pH; the two chains remain linked via two disulfide bonds (PubMed:25156943). Also proteolytically processed by TMPRSS6, several fragments being released in the extracellular space; regulates HJV activity in BMP signaling and thefore iron homeostasis (PubMed:18976966, PubMed:25156943).
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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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