Mouse anti-Human CYPOR / POR Monoclonal Antibody | anti-CYPOR / POR antibody
Anti-CYPOR / POR Antibody (clone 6C4) IHC-plus
IF (1:50 - 1:100)
IHC-P (1:50 - 1:100)
WB (1:2000)
Immunohistochemistry (IHC)
(Anti-CYPOR / POR antibody IHC staining of human liver. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval.)
Immunofluorescence (IF)
(Immunofluorescent staining of HeLa cells using anti-POR mouse monoclonal antibody.)
Immunofluorescence (IF)
(Anti-POR mouse monoclonal antibody immunofluorescent staining of COS7 cells transiently transfected by pCMV6-ENTRY POR.)
Western Blot (WB)
(HEK293T cells were transfected with the pCMV6-ENTRY control (Left lane) or pCMV6-ENTRY POR (Right lane) cDNA for 48 hrs and lysed. Equivalent amounts of cell lysates (5 ug per lane) were separated by SDS-PAGE and immunoblotted with anti-POR.)
Flow Cytometry (FC/FACS)
(Flow cytometry of HeLa cells, using anti-POR antibody (Red), compared to a nonspecific negative control antibody (Blue).)
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; integral to membrane; intracellular membrane-bound organelle; membrane; mitochondrion
Molecular Function: [methionine synthase] reductase activity; cytochrome-b5 reductase activity; electron carrier activity; enzyme binding; FAD binding; FMN binding; hydrolase activity; iron ion binding; iron-cytochrome-c reductase activity; NADP binding; NADPH-hemoprotein reductase activity; nitric oxide dioxygenase activity; protein binding
Biological Process: carnitine metabolic process; fatty acid oxidation; flavonoid metabolic process; internal peptidyl-lysine acetylation; negative regulation of caspase activity; negative regulation of lipase activity; nitrate catabolic process; nitric oxide catabolic process; positive regulation of cholesterol biosynthetic process; positive regulation of chondrocyte differentiation; positive regulation of monooxygenase activity; positive regulation of smoothened signaling pathway; response to drug; response to nutrient
Disease: Antley-bixler Syndrome With Genital Anomalies And Disordered Steroidogenesis; Antley-bixler Syndrome Without Genital Anomalies Or Disordered Steroidogenesis; Disordered Steroidogenesis Due To Cytochrome P450 Oxidoreductase Deficiency