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货号: YP-mAb-02559
促销价:

产品介绍

反应种属
Human
应用范围
WB
抗体类型
单克隆抗体
基因名称(Gene Name)
CYP11A1
蛋白名称
Cholesterol side-chain cleavage enzyme mitochondrial
分子量(DA)
60kD
免疫原
The antiserum was produced against synthesized peptide derived from human Cytochrome P450 11A1. AA range:412-461
特异性
CYP11A1 Monoclonal Antibody detects endogenous levels of CYP11A1 protein.
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
来源
Monoclonal, Mouse,IgG
稀释比例
Western Blot: 1/500 - 1/2000
纯化工艺
The antibody was affinity-purified from mouse antiserum by affinity-chromatography using epitope-specific immunogen.
浓度
1 mg/ml
储存
-20°C/1 year
其他名称
CYP11A1; CYP11A; Cholesterol side-chain cleavage enzyme; mitochondrial; CYPXIA1; Cholesterol desmolase; Cytochrome P450 11A1; Cytochrome P450(scc)
背景
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

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