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Mouse Anti-HJV Recombinant Antibody (A183) (CBMAB-AP2954LY)


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Summary

Host Animal
Mouse
Specificity
Human, Pig, Rat
Clone
A183
Antibody Isotype
IgG2b, κ
Application
ICC, IHC, IP, WB

Basic Information

Immunogen
Lys234~Ser416
Specificity
Human, Pig, Rat
Antibody Isotype
IgG2b, 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
Affinity purity
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
hemojuvelin BMP co-receptor
Entrez Gene ID
Human148738
Rat310681
Pig100155778
UniProt ID
HumanQ6ZVN8
RatQ8N7M5
PigA0A287AYZ9
Alternative Names
HFE2, HFE2A, HJV, JH, RGMC, hemochromatosis type 2(Juvenile), Haemojuvelin, RGM domain family member C
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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