Detection Method:Sandwich
Test principle:This assay employs a two-site sandwich ELISA to quantitate ABCC6 in samples. An antibody specific for ABCC6 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyABCC6 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for ABCC6 is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of ABCC6 bound in the initial step. The color development is stopped and the intensity of the color is measured.
Product Overview:Multidrug resistance-associated protein 6 is member of the superfamily of ATP-binding cassette (ABC) transporters.ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White).
This protein is a member of the MRP subfamily which is involved in multidrug resistance.Defects in ABCC6 are the cause of pseudoxanthoma elasticum (PXE). PXE is a disorder characterized by calcification of elastic fibers in skin, arteries and retina that results in dermal lesions with associated laxity and loss of elasticity, arterial insufficiency and retinal hemorrhages leading to macular degeneration.