NP_058982.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
60,586 Da
NCBI Official Full Name
cholesterol side-chain cleavage enzyme, mitochondrial
NCBI Official Synonym Full Names
cytochrome P450, family 11, subfamily a, polypeptide 1
NCBI Protein Information
cholesterol side-chain cleavage enzyme, mitochondrial
UniProt Protein Name
Cholesterol side-chain cleavage enzyme, mitochondrial
UniProt Synonym Protein Names
CYPXIA1; Cholesterol desmolase; Cytochrome P450 11A1; Cytochrome P450(scc)
UniProt Synonym Gene Names
UniProt Entry Name
CP11A_RAT
NCBI Summary for CYP11A1
monooxygenase that catalyzes synthesis of cholesterol and steroids [RGD, Feb 2006]
UniProt Comments for CYP11A1
CYP11A1: Catalyzes the side-chain cleavage reaction of cholesterol to pregnenolone. Defects in CYP11A1 are the cause of adrenal insufficiency congenital with 46,XY sex reversal (AICSR). A rare disorder that can present as acute adrenal insufficiency in infancy or childhood. ACTH and plasma renin activity are elevated and adrenal steroids are inappropriately low or absent; the 46,XY patients have female external genitalia, sometimes with clitoromegaly. The phenotypic spectrum ranges from prematurity, complete underandrogenization, and severe early-onset adrenal failure to term birth with clitoromegaly and later-onset adrenal failure. Patients with congenital adrenal insufficiency do not manifest the massive adrenal enlargement typical of congenital lipoid adrenal hyperplasia. Belongs to the cytochrome P450 family.
Protein type: EC 1.14.15.6; Mitochondrial; Lipid Metabolism - C21-steroid hormone; Oxidoreductase
Cellular Component: mitochondrial crista; mitochondrion; mitochondrial inner membrane; perikaryon
Molecular Function: cholesterol monooxygenase (side-chain-cleaving) activity; iron ion binding; cholesterol binding; heme binding
Biological Process: response to alkaloid; response to peptide hormone stimulus; maternal process involved in pregnancy; response to cAMP; dibenzo-p-dioxin metabolic process; response to insecticide; response to L-ascorbic acid; response to organic cyclic substance; estrogen biosynthetic process; response to salt stress; response to organic substance; response to antibiotic; organic acid metabolic process; response to vitamin E; Leydig cell differentiation; biphenyl metabolic process; response to gamma radiation; cerebellum development; response to nutrient; response to corticosterone stimulus; response to drug; cholesterol metabolic process; progesterone biosynthetic process; hippocampus development; phthalate metabolic process; male gonad development; mating behavior; response to amino acid stimulus; Schwann cell differentiation; response to genistein; response to cadmium ion; response to hydrogen peroxide; response to estrogen stimulus; response to steroid hormone stimulus; C21-steroid hormone biosynthetic process; response to ionizing radiation; steroid biosynthetic process; granulosa cell differentiation; phenol metabolic process
Research Articles on CYP11A1
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Products associated with CYP11A1 sirna
Pathways associated with CYP11A1 sirna
Diseases associated with CYP11A1 sirna
Organs/Tissues associated with CYP11A1 sirna
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