Human Cytochrome P450 Reductase (CPR) ELISA Kit | CPR elisa kit
Human Cytochrome P450 Reductase (CPR) ELISA Kit
To minimize extra influence on the performance, operation procedures and lab conditions, especially room temperature, air humidity, incubator temperature should be strictly controlled. It is also strongly suggested that the whole assay is performed by the same operator from the beginning to the end.
Principle of the Assay: The microplate provided in this kit has been pre-coated with an antibody specific to CPN1. Standards or samples are then added to the appropriate microplate wells with a biotin-conjugated antibody specific to CPN1. Next, Avidin conjugated to Horseradish Peroxidase (HRP) is added to each microplate well and incubated. After TMB substrate solution is added, only those wells that contain CPN1, biotin-conjugated antibody and enzyme-conjugated Avidin will exhibit a change in color. The enzyme-substrate reaction is terminated by the addition of sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450nm +/- 10nm. The concentration of CPN1 in the samples is then determined by comparing the O.D. of the samples to the standard curve.
NCBI and Uniprot Product Information
NCBI Description
This gene encodes an endoplasmic reticulum membrane oxidoreductase with an FAD-binding domain and a flavodoxin-like domain. The protein binds two cofactors, FAD and FMN, which allow it to donate electrons directly from NADPH to all microsomal P450 enzymes. Mutations in this gene have been associated with various diseases, including apparent combined P450C17 and P450C21 deficiency, amenorrhea and disordered steroidogenesis, congenital adrenal hyperplasia and Antley-Bixler syndrome. [provided by RefSeq, Jul 2008]
Uniprot Description
POR: This enzyme is required for electron transfer from NADP to cytochrome P450 in microsomes. It can also provide electron transfer to heme oxygenase and cytochrome B5. Defects in POR are the cause of Antley-Bixler syndrome with genital anomalies and disordered steroidogenesis (ABS1). A disease characterized by the association of Antley-Bixler syndrome with steroidogenesis defects and abnormal genitalia. Antley-Bixler syndrome is characterized by craniosynostosis, radiohumeral synostosis present from the perinatal period, midface hypoplasia, choanal stenosis or atresia, femoral bowing and multiple joint contractures. Defects in POR are the cause of disordered steroidogenesis due to cytochrome P450 oxidoreductase deficiency (DISPORD). A disorder resulting in a rare variant of congenital adrenal hyperplasia, with apparent combined P450C17 and P450C21 deficiency and accumulation of steroid metabolites. Affected girls are born with ambiguous genitalia, but their circulating androgens are low and virilization does not progress. Conversely, affected boys are sometimes born undermasculinized. Boys and girls can present with bone malformations, in some cases resembling the pattern seen in patients with Antley-Bixler syndrome.
Protein type: EC 1.6.2.4; Oxidoreductase
Chromosomal Location of Human Ortholog: 7q11.2
Cellular Component: endoplasmic reticulum membrane; mitochondrion; membrane; intracellular membrane-bound organelle
Molecular Function: protein binding; enzyme binding; FAD binding; electron carrier activity; NADPH-hemoprotein reductase activity; hydrolase activity; FMN binding; nitric oxide dioxygenase activity; cytochrome-b5 reductase activity; iron ion binding; NADP binding; iron-cytochrome-c reductase activity
Biological Process: response to drug; nitric oxide catabolic process; nitrate catabolic process; positive regulation of chondrocyte differentiation; internal peptidyl-lysine acetylation; flavonoid metabolic process; positive regulation of monooxygenase activity; positive regulation of cholesterol biosynthetic process; positive regulation of smoothened signaling pathway; carnitine metabolic process; fatty acid oxidation; negative regulation of caspase activity; negative regulation of lipase activity; response to nutrient
Disease: Disordered Steroidogenesis Due To Cytochrome P450 Oxidoreductase Deficiency; Antley-bixler Syndrome With Genital Anomalies And Disordered Steroidogenesis; Antley-bixler Syndrome Without Genital Anomalies Or Disordered Steroidogenesis