ENTRY EC 1.14.15.6
NAME Cholesterol monooxygenase (side-chain-cleaving)
Cholesterol desmolase
Cytochrome P-450SCC
CLASS Oxidoreductases
Acting on paired donors with incorporation of molecular oxygen
With a reduced iron-sulfur protein as one donor, and
incorporation of one atom of oxygen
SYSNAME Cholesterol,reduced-adrenal-ferredoxin:oxygen oxidoreductase
(side-chain-cleaving)
REACTION Cholesterol + Reduced adrenal ferredoxin + O2 = Pregnenolone +
4-Methylpentanal + Oxidized adrenal ferredoxin + H2O
SUBSTRATE Cholesterol
Reduced adrenal ferredoxin
O2
PRODUCT Pregnenolone
4-Methylpentanal
Oxidized adrenal ferredoxin
H2O
COFACTOR Heme
COMMENT A heme-thiolate protein. The reaction proceeds in three stages,
with hydroxylation at C-20 and C-22 preceding scission of the
side-chain at C-20.
PATHWAY PATH: MAP00140 C21-Steroid hormone metabolism
DISEASE MIM: 118485 Cytochrome P450, subfamily XIA (cholesterol side chain
cleavage); Polycystic ovary syndrome with
hyperandrogenemia (2)
MOTIF PS: PS00086 F-[SGNH]-x-[GD]-x-[RHPT]-x-C-[LIVMFAP]-[GAD]
GENES HSA: CYP11A(Hs.76205)
STRUCTURES PDB: 1SCC
DBLINKS University of Geneva ENZYME DATA BANK: 1.14.15.6
WIT (What Is There) Metabolic Reconstruction: 1.14.15.6
SCOP (Structural Classificaion of Proteins): 1.14.15.6
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