Description
Description: This assay employs a two-site sandwich ELISA to quantitate MPV17L2 in samples. An antibody specific for MPV17L2 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any MPV17L2 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for MPV17L2 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 MPV17L2 bound in the initial step. The color development is stopped and the intensity of the color is measured.
Overview: MPV17L2 Belongs to the peroxisomal membrane protein PXMP2/4 family. MPV17 encodes a mitochondrial inner membrane protein that is implicated in the metabolism of reactive oxygen species. Mutations in this gene have been associated with the hepatocerebral form of MDDS.The Mpv17 strain of mice carries a transgenically produced retroviral insert in its genome. The integration prevents the expression of the Mpv17 gene, leading to the development of glomerulosclerosis in mice homozygous for the integration. Physiologically and histologically, the phenotype of the kidney disease resembles human glomerulosclerosis. The mice demonstrate nephrotic syndrome at an early age.Sequence analysis demonstrated over 90% identity to the mouse Mpv17 gene in the region coding for a protein of 176 amino acids and unknown function