Anti-POR Antibody Picoband® (monoclonal, 7F5) Fluoro488 Conjugated

POR is a membrane-boundenzyme required for electron transfer from NADPH to cytochrome P450 in the endoplasmic reticulum of theeukaryotic cell. The 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 ly 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.

A synthetic peptide corresponding to a sequence at the C-terminus of human POR, different from the related mouse and rat sequences by five amino acids.

Expressed in a defined zone of basal keratinocytes in the deep outer root sheath of hair follicles. Also observed in sweat gland and mammary gland ductal and secretory cells, bile ducts, gastrointestinal tract, bladder urothelium, oral epithelia, esophagus, ectocervical epithelium (at protein level) . Expressed in epidermal basal cells, in nipple epidermis and a defined region of the hair follicle. Also seen in a subset of vascular wall cells in both the veins and artery of human umbilical cord, and in umbilical cord vascular smooth muscle. Observed in muscle fibers accumulating in the costameres of myoplasm at the sarcolemma in structures that contain dystrophin and spectrin.

Involved in the organization of myofibers. Together with KRT8, helps to link the contractile apparatus to dystrophin at the costameres of striated muscle.

1. Adachi, M., Asakura, Y., Matsuo, M., Yamamoto, T., Hanaki, K., Arlt, W. POR R457H is a global founder mutation causing Antley-Bixler syndrome with autosomal recessive trait. (Letter) Am. J. Med. Genet. 140A: 633-635, 2006. 2. Miller, W. L. Congenital adrenal hyperplasia. (Letter) New Eng. J. Med. 314: 1321-1322, 1986. 3. Shephard, E. A., Phillips, I. R., Santisteban, I., West, L. F., Palmer, C. N., Ashworth, A., Povey, S. Isolation of a human cytochrome P-450 reductase cDNA clone and localization of the corresponding gene to chromosome 7q11.2. Ann. Hum. Genet. 53: 291-301, 1989.