Rabbit anti-Human CYP11A1 Polyclonal Antibody

Uniprot Id

P05108

Target Species

Human

Target Name

CYP11A1

Target Full Name

Cholesterol side-chain cleavage enzyme, mitochondrial

Target Function

A cytochrome P450 monooxygenase that catalyzes the side-chain hydroxylation and cleavage of cholesterol to pregnenolone, the precursor of most steroid hormones. Catalyzes three sequential oxidation reactions of cholesterol, namely the hydroxylation at C22 followed with the hydroxylation at C20 to yield 20R,22R-hydroxycholesterol that is further cleaved between C20 and C22 to yield the C21-steroid pregnenolone and 4-methylpentanal. Mechanistically, uses molecular oxygen inserting one oxygen atom into a substrate and reducing the second into a water molecule. Two electrons are provided by NADPH via a two-protein mitochondrial transfer system comprising flavoprotein FDXR (adrenodoxin/ferredoxin reductase) and nonheme iron-sulfur protein FDX1 or FDX2 (adrenodoxin/ferredoxin).

Target Involvement

Adrenal insufficiency, congenital, with 46,XY sex reversal (AICSR)

Target Subcellular Location

Mitochondrion inner membrane; Peripheral membrane protein.

Target Protein Families

Cytochrome P450 family

Target Research Area

Metabolism

Target Synonyms

Cholesterol 20 22 desmolase; Cholesterol desmolase; Cholesterol monooxygenase (side chain cleaving); Cholesterol side chain cleavage enzyme; Cholesterol side chain cleavage enzyme mitochondrial; Cholesterol side-chain cleavage enzyme; CP11A_HUMAN; CYP11A; CYP11A1; CYPXIA1; Cytochrome P450 11A1; Cytochrome P450 11A1 mitochondrial; Cytochrome P450 family 11 subfamily A polypeptide 1; Cytochrome P450 subfamily XIA; Cytochrome P450(scc); Cytochrome P450C11A1; mitochondrial; P450SCC; Steroid 20 22 lyase

Target Background

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.