免疫原
The antiserum was produced against synthesized peptide derived from human Cytochrome P450 11A1. AA range:412-461
特异性
CYP11A1 Polyclonal Antibody detects endogenous levels of CYP11A1 protein.
来源
Polyclonal, Rabbit,IgG
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
Western Blot: 1/500 - 1/2000. ELISA: 1/5000. Not yet tested in other applications.
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
背景（Background）
cytochrome P450 family 11 subfamily A member 1(CYP11A1) Homo sapiens This gene encodes a member of the cytochrome P450 superfamily of enzymes. The cytochrome P450 proteins are monooxygenases which catalyze many reactions involved in drug metabolism and synthesis of cholesterol, steroids and other lipids. This protein localizes to the mitochondrial inner membrane and catalyzes the conversion of cholesterol to pregnenolone, the first and rate-limiting step in the synthesis of the steroid hormones. Two transcript variants encoding different isoforms have been found for this gene. The cellular location of the smaller isoform is unclear since it lacks the mitochondrial-targeting transit peptide. [provided by RefSeq, Jul 2008],
功能
catalytic activity:Cholesterol + reduced adrenal ferredoxin + O(2) = pregnenolone + 4-methylpentanal + oxidized adrenal ferredoxin + H(2)O.,cofactor:Heme group.,disease:Defects in CYP11A1 are a cause of congenital adrenal insufficiency (CAI).,disease:Defects in CYP11A1 are a cause of congenital lipoid adrenal hyperplasia (CLAH) [MIM:201710]; also called lipoid CAH. CLAH is the most severe form of adrenal hyperplasia. This autosomal recessive and potentially lethal condition includes the onset of profound adrenocortical insufficiency shortly after birth, hyperpigmentation reflecting increased production of pro-opiomelanocortin, elevated plasma renin activity as a consequence of reduced aldosterone synthesis, and male pseudohermaphroditism resulting from deficient fetal testicular testosterone synthesis. CLAH is a rare disease, except in Japan and Korea where it accounts for a significant
蛋白名称
Cholesterol side-chain cleavage enzyme mitochondrial
其他名称
CYP11A1; CYP11A; Cholesterol side-chain cleavage enzyme; mitochondrial; CYPXIA1; Cholesterol desmolase; Cytochrome P450 11A1; Cytochrome P450(scc)
Fields
>>Steroid hormone biosynthesis;>>Metabolic pathways;>>Ovarian steroidogenesis;>>Aldosterone synthesis and secretion;>>Cortisol synthesis and secretion;>>Cushing syndrome
细胞定位
Mitochondrion inner membrane ; Peripheral membrane protein . Localizes to the matrix side of the mitochondrion inner membrane. .
组织表达
Brain,Choriocarcinoma,Placenta,
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