Names & Taxonomy
- Uniprot ID:
- Q02318
- Entry Name:
- CP27A_HUMAN
- Status:
- reviewed
- Protein Names:
- Sterol 26-hydroxylase, mitochondrial (EC 1.14.15.15) (5-beta-cholestane-3-alpha,7-alpha,12-alpha-triol 27-hydroxylase) (Cytochrome P-450C27/25) (Cytochrome P450 27) (Sterol 27-hydroxylase) (Vitamin D(3) 25-hydroxylase)
- Gene Names:
- CYP27A1 CYP27
- Gene Names Primary:
- CYP27A1
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 531
- Sequence:
- MAALGCARLRWALRGAGRGLCPHGARAKAAIPAALPSDKATGAPGAGPGVRRRQRSLEEIPRLGQLRFFFQLFVQGYALQLHQLQVLYKAKYGPMWMSYLGPQMHVNLASAPLLEQVMRQEGKYPVRNDMELWKEHRDQHDLTYGPFTTEGHHWYQLRQALNQRLLKPAEAALYTDAFNEVIDDFMTRLDQLRAESASGNQVSDMAQLFYYFALEAICYILFEKRIGCLQRSIPEDTVTFVRSIGLMFQNSLYATFLPKWTRPVLPFWKRYLDGWNAIFSFGKKLIDEKLEDMEAQLQAAGPDGIQVSGYLHFLLASGQLSPREAMGSLPELLMAGVDTTSNTLTWALYHLSKDPEIQEALHEEVVGVVPAGQVPQHKDFAHMPLLKAVLKETLRLYPVVPTNSRIIEKEIEVDGFLFPKNTQFVFCHYVVSRDPTAFSEPESFQPHRWLRNSQPATPRIQHPFGSVPFGYGVRACLGRRIAELEMQLLLARLIQKYKVVLAPETGELKSVARIVLVPNKKVGLQFLQRQC
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion membrane.
Function
- Function:
- Catalyzes the first step in the oxidation of the side chain of sterol intermediates; the 27-hydroxylation of 5-beta-cholestane-3-alpha,7-alpha,12-alpha-triol. Has also a vitamin D3-25-hydroxylase activity.
- Pathway:
- Hormone biosynthesis; cholecalciferol biosynthesis.
- Catalytic Activity:
- 5-beta-cholestane-3-alpha,7-alpha,12-alpha-triol + 6 reduced adrenodoxin + 6 H(+) + 3 O(2) = (25R)-3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestan-26-oate + 6 oxidized adrenodoxin + 4 H(2)O.
- Cofactor:
- COFACTOR: Name=heme; Xref=ChEBI:CHEBI:30413;
- Cross Reference Drug Bank:
- DB06777 DB00169 DB06410 DB00153 DB00082
- Gene Ontology Go:
- mitochondrial inner membrane
mitochondrial matrix
cholestanetriol 26-monooxygenase activity
heme binding
iron ion binding
steroid hydroxylase activity
bile acid biosynthetic process
bile acid metabolic process
small molecule metabolic process
sterol metabolic process
xenobiotic metabolic process - Gene Ontology Biological Process:
- bile acid biosynthetic process
bile acid metabolic process
small molecule metabolic process
sterol metabolic process
xenobiotic metabolic process - Gene Ontology Molecular Function:
- cholestanetriol 26-monooxygenase activity
heme binding
iron ion binding
steroid hydroxylase activity - Gene Ontology Cellular Component:
- mitochondrial inner membrane
mitochondrial matrix - Keywords:
- 3D-structure
Acetylation
Cataract
Complete proteome
Disease mutation
Heme
Iron
Membrane
Metal-binding
Mitochondrion
Monooxygenase
NADP
Oxidoreductase
Polymorphism
Reference proteome
Transit peptide