ENTRY EC 1.14.15.4
NAME Steroid 11beta-monooxygenase
Steroid 11beta-hydroxylase
Steroid 11beta/18-hydroxylase
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 Steroid,reduced-adrenal-ferredoxin:oxygen oxidoreductase
(11-hydroxylating)
REACTION a Steroid + Reduced adrenal ferredoxin + O2 =
an 11beta-Hydroxysteroid + Oxidized adrenal ferredoxin + H2O
SUBSTRATE Steroid
Reduced adrenal ferredoxin
O2
18-Hydroxycorticosterone
PRODUCT 11beta-Hydroxysteroid
Oxidized adrenal ferredoxin
H2O
Aldosterone
COFACTOR Heme
COMMENT A heme-thiolate protein (P-450). Also hydroxylates steroids at
the 18-position, and converts 18-hydroxycorticosterone into
aldosterone. Formerly EC 1.14.1.6 and 1.99.1.7.
PATHWAY PATH: MAP00140 C21-Steroid hormone metabolism
PATH: MAP00150 Androgen and estrogen metabolism
DISEASE MIM: 124080 Cytochrome P450, subfamily XIB, polypeptide-2; CMO II
deficiency (3)
MIM: 202010 Cytochrome P450, subfamily XIB, polypeptide-1;
11-beta-hydroxylase;; Adrenal hyperplasia, congenital,
due to 11-beta-hydroxylase deficiency (3);
Aldosteronism, glucocorticoid-remediable (3)
MOTIF PS: PS00086 F-[SGNH]-x-[GD]-x-[RHPT]-x-C-[LIVMFAP]-[GAD]
GENES HSA: CYP11B1(CYP11B) CYP11B2(CYP11B)
DBLINKS University of Geneva ENZYME DATA BANK: 1.14.15.4
WIT (What Is There) Metabolic Reconstruction: 1.14.15.4
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