Description
This Human Hemojuvelin ELISA Kit is intended for quantitative detection of human Hemojuvelin in cell culture supernates, cell lysates, serum and plasma (heparin, EDTA). Strip well format. Reagents for up to 96 tests.
This human Hemojuvelin ELISA Kit was based on standard sandwich enzyme-linked immune-sorbent assay technology. A monoclonal antibody from mouse specific for Hemojuvelin has been precoated onto 96-well plates. Standards (Expression system for standard: NSO, Immunogen sequence: Q36-D400) and test samples are added to the wells, a biotinylated detection polyclonal antibody from goat specific for Hemojuvelin is added subsequently and then followed by washing with PBS or TBS buffer. Avidin-Biotin-Peroxidase Complex was added and unbound conjugates were washed away with PBS or TBS buffer. HRP substrate TMB was used to visualize HRP enzymatic reaction. TMB was catalyzed by HRP to produce a blue color product that changed into yellow after adding acidic stop solution. The density of yellow is proportional to the human Hemojuvelin amount of sample captured in plate.
The capture antibody is a monoclonal antibody from mouse, the detection antibody is a biotinylated polyclonal antibody from goat. Expression system for standard: 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