Human Hemojuvelin/RGM-C/HJV ELISA Kit PicoKine® | Boster EK1369 product information

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product summary

company name :

Boster

product type :

ELISA/assay

product name :

Human Hemojuvelin/RGM-C/HJV ELISA Kit PicoKine®

catalog :

EK1369

quantity :

96 wells/kit, with removable strips.

product information

SKU :

EK1369

Product Name :

Human Hemojuvelin/RGM-C/HJV ELISA Kit PicoKine®

Size :

96 wells/kit, with removable strips.

Description :

Human Hemojuvelin/RGM-C/HJV ELISA Kit PicoKine® (96 Tests). Quantitate Human HJV in cell culture supernatants, cell lysates, serum and plasma (heparin, EDTA). Sensitivity: 15pg/ml.

Reactivity :

Human

Application :

ELISA

Gene Name :

HJV

Uniprot ID :

Q6ZVN8

Immunogen :

Expression system for standard: NS0; Immunogen sequence: Q36-D400

Cross-reactivity :

There is no detectable cross-reactivity with other relevant proteins.

Assay Range :

156 pg/ml - 10,000 pg/ml

Sensitivity :

<15 pg/ml

Sample Type :

cell culture supernatants, cell lysates, serum and plasma (heparin, EDTA)

Capture/Detection Antibodies :

a monoclonal antibody from mouse, a biotinylated polyclonal antibody from goat

Storage :

Store at 4°C for 6 months, at -20°C for 12 months. Avoid multiple freeze-thaw cycles (Ships with gel ice, can store for up to 3 days in room temperature. Freeze upon receiving.)

Gene Full Name :

Hemojuvelin

Synonyms :

Hemojuvelin;Hemochromatosis type 2 protein;RGM domain family member C;HFE2;HJV, RGMC;

Protein Name :

Hemojuvelin

Molecular Weight :

45080 MW

Protein Function :

Acts as a bone morphogenetic protein (BMP) coreceptor. Through enhancement of BMP signaling regulates hepcidin (HAMP) expression and regulates iron homeostasis.

Subcellular Localization :

Cell membrane ; Lipid-anchor, GPI-anchor . Also released in the extracellular space.

Tissue Specificity :

Adult and fetal liver, heart, and skeletal muscle.

Background :

Hemojuvelin (HJV/RGMc/HFE2) is a membrane-bound and soluble protein in mammals. It is a member of the repulsive guidance molecule family of proteins. In humans, the hemojuvelin protein is encoded by the HFE2 gene. 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. Two uORFs in the 5' UTR negatively regulate the expression and activity of the encoded protein. Alternatively spliced transcript variants encoding different isoforms have been identified for this gene. 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.