Synthesized peptide derived from human protein . at AA range: 280-360

C11B2 Polyclonal Antibody detects endogenous levels of protein.

CYP11B2

Cytochrome P450 11B2, mitochondrial (Aldosterone synthase) (ALDOS) (Aldosterone-synthesizing enzyme) (CYPXIB2) (Cytochrome P-450Aldo) (Cytochrome P-450C18) (Steroid 18-hydroxylase)

cytochrome P450 family 11 subfamily B member 2(CYP11B2) 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. The enzyme has steroid 18-hydroxylase activity to synthesize aldosterone and 18-oxocortisol as well as steroid 11 beta-hydroxylase activity. Mutations in this gene cause corticosterone methyl oxidase deficiency. [provided by RefSeq, Jul 2008],

Catalytic activity:A steroid + reduced adrenal ferredoxin + O(2) = an 11-beta-hydroxysteroid + oxidized adrenal ferredoxin + H(2)O.,Catalytic activity:Corticosterone + reduced adrenal ferredoxin + O(2) = 18-hydroxycorticosterone + oxidized adrenal ferredoxin + H(2)O.,cofactor:Heme group.,Disease:An anti-Lepore-type fusion of the CYP11B2 and CYP11B1 genes is a cause of glucocorticoid-remediable aldosteronism (GRA) [MIM:103900].,Disease:Defects in CYP11B2 are the cause of corticosterone methyloxidase type 1 deficiency (CMO-1 deficiency) [MIM:203400]; also called aldosterone deficiency due to defect in 18-hydroxylase or aldosterone deficiency I. CMO-1 deficiency is an autosomal recessive disorder of aldosterone biosynthesis. There are two biochemically different forms of selective aldosterone deficiency be termed corticosterone methyloxidase (CMO) deficiency type 1 and type 2. In CMO-1 deficiency, aldosterone is undetectable in plasma, while its immediate precursor, 18-hydroxycorticosterone, is low or normal.,Disease:Defects in CYP11B2 are the cause of corticosterone methyloxidase type 2 deficiency (CMO-2 deficiency) [MIM:610600]. CMO-2 is an autosomal recessive disorder of aldosterone biosynthesis. In CMO-2 deficiency, aldosterone can be low or normal, but at the expense of increased secretion of 18-hydroxycorticosterone. Consequently, patients have a greatly increased ratio of 18-hydroxycorticosterone to aldosterone and a low ratio of corticosterone to 18-hydroxycorticosterone in serum.,Function:Preferentially catalyzes the conversion of 11-deoxycorticosterone to aldosterone via corticosterone and 18-hydroxycorticosterone.,online information:CYP11B2 entry,similarity:Belongs to the cytochrome P450 family.,

Mitochondrion inner membrane ; Peripheral membrane protein .

Adrenal gland,Blood,

>>Steroid hormone biosynthesis ;
>>Metabolic pathways ;
>>Aldosterone synthesis and secretion