Reaction: CYP27A1 27-hydroxylates 5bCHOL3a,7a,12a-triol
CYP27A1, a mitochondrial matrix sterol hydroxylase, catalyses the 27-hydroxylation of side-chains of sterol intermediates (Cali et al. 1991). A specific reaction is the 27-hydroxylation of 5beta-cholestan-3alpha, 7alpha, 12alpha-triol (5bCHOL3a,7a,12a-triol) to form 5beta-cholestan-3alpha, 7alpha, 12alpha, 27-tetrol (5bCHOL3a,7a,12a, 27-tetrol) (Pikuleva et al. 1998). Defects in CYP27A1 can cause Cerebrotendinous xanthomatosis (CTX; MIM:213700), a rare sterol storage disorder characterised by progressive neurologic dysfunction, premature atherosclerosis and cataracts (Cali et al. 1991b).
Reaction - small molecule participants:
5bCHOL3a,7a,12a,27-tetrol [mitochondrial matrix]
NADP+ [mitochondrial matrix]
H2O [mitochondrial matrix]
O2 [mitochondrial matrix]
5bCHOL3a,7a,12a-triol [mitochondrial matrix]
H+ [mitochondrial matrix]
NADPH [mitochondrial matrix]
Reactome.org reaction link: R-HSA-191999
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Reaction input - small molecules:
dioxygen
5beta-cholestane-3alpha,7alpha,12alpha-triol
hydron
NADPH
Reaction output - small molecules:
5beta-cholestane-3alpha,7alpha,12alpha,26-tetrol
NADP(+)
water
Reactome.org link: R-HSA-191999