免疫原
The antiserum was produced against synthesized peptide derived from the N-terminal region of human CYP11B1/2. AA range:61-110
特异性
CYP11B1/2 Polyclonal Antibody detects endogenous levels of CYP11B1/2 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: 1/20000. 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 B member 1(CYP11B1) 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 is involved in the conversion of progesterone to cortisol in the adrenal cortex. Mutations in this gene cause congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency. Transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Jul 2008],
功能
catalytic activity:A steroid + reduced adrenal ferredoxin + O(2) = an 11-beta-hydroxysteroid + oxidized adrenal ferredoxin + H(2)O.,cofactor:Heme group.,disease:An anti-Lepore-type fusion of the CYP11B1 and CYP11B2 genes is a cause of glucocorticoid-remediable aldosteronism (GRA) [MIM:103900].,disease:Defects in CYP11B1 are the cause of adrenal hyperplasia type 4 (AH4) [MIM:202010]. AH4 is a form of congenital adrenal hyperplasia, a common recessive disease due to defective synthesis of cortisol. Congenital adrenal hyperplasia is characterized by androgen excess leading to ambiguous genitalia in affected females, rapid somatic growth during childhood in both sexes with premature closure of the epiphyses and short adult stature. Four clinical types: "salt wasting" (SW, the most severe type), "simple virilizing" (SV, less severely affected patients), with normal aldosterone biosynthesis, "
基因名称（Gene Name）
CYP11B1/CYP11B2
蛋白名称
Cytochrome P450 11B1 mitochondrial/Cytochrome P450 11B2 mitochondrial
其他名称
CYP11B1; S11BH; Cytochrome P450 11B1, mitochondrial; CYPXIB1; Cytochrome P-450c11; Cytochrome P450C11; Steroid 11-beta-hydroxylase; CYP11B2;Cytochrome P450 11B2, mitochondrial; Aldosterone synthase; ALDOS; Aldosterone-synthesizing enzyme; CYPXIB2; Cytochrome P-450Aldo; Cytochrome P-450C18; Steroid 18-hydroxylase
Fields
>>Steroid hormone biosynthesis;>>Metabolic pathways;>>Cortisol synthesis and secretion;>>Cushing syndrome
细胞定位
Mitochondrion inner membrane ; Peripheral membrane protein .
组织表达
Adrenal gland,PCR rescued clones,Peripheral blood,
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