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

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Basic Information

Host Animal
Mouse
Clone
A183
Application
ICC, IHC, IP, WB
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.
More Infomation

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
MAB995Hu22
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).

Novikov, N., Buch, A., Yang, H., Tuller, S., Andruk, M., Chan, K., ... & Savage, W. (2022). DISC-0974, an anti-hemojuvelin antibody, reduces hepcidin and mobilizes iron in healthy volunteers. Blood, 140(Supplement 1), 5339-5340.

Tippairote, T., Bjørklund, G., Peana, M., & Roytrakul, S. (2021). The proteomics study of compounded HFE/TF/TfR2/HJV genetic variations in a Thai family with iron overload, chronic anemia, and motor neuron disorder. Journal of Molecular Neuroscience, 71(3), 545-555.

Pettinato, M., Aghajan, M., Olivari, V., Dulja, A., Nai, A., Bordini, J., ... & Silvestri, L. (2019). Targeting the BMP Receptor Inhibitor FKBP12 to Upregulate Hepcidin in Wild-Type and Hemojuvelin-Hemochromatosis Models. Blood, 134, 955.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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Mouse Anti-HJV Recombinant Antibody (A181) (CAT#: CBMAB-AP2955LY)

Isotype control

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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