Names & Taxonomy
- Uniprot ID:
- P05108
- Entry Name:
- CP11A_HUMAN
- Status:
- reviewed
- Protein Names:
- Cholesterol side-chain cleavage enzyme, mitochondrial (EC 1.14.15.6) (CYPXIA1) (Cholesterol desmolase) (Cytochrome P450 11A1) (Cytochrome P450(scc))
- Gene Names:
- CYP11A1 CYP11A
- Gene Names Primary:
- CYP11A1
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 521
- Sequence:
- MLAKGLPPRSVLVKGCQTFLSAPREGLGRLRVPTGEGAGISTRSPRPFNEIPSPGDNGWLNLYHFWRETGTHKVHLHHVQNFQKYGPIYREKLGNVESVYVIDPEDVALLFKSEGPNPERFLIPPWVAYHQYYQRPIGVLLKKSAAWKKDRVALNQEVMAPEATKNFLPLLDAVSRDFVSVLHRRIKKAGSGNYSGDISDDLFRFAFESITNVIFGERQGMLEEVVNPEAQRFIDAIYQMFHTSVPMLNLPPDLFRLFRTKTWKDHVAAWDVIFSKADIYTQNFYWELRQKGSVHHDYRGILYRLLGDSKMSFEDIKANVTEMLAGGVDTTSMTLQWHLYEMARNLKVQDMLRAEVLAARHQAQGDMATMLQLVPLLKASIKETLRLHPISVTLQRYLVNDLVLRDYMIPAKTLVQVAIYALGREPTFFFDPENFDPTRWLSKDKNITYFRNLGFGWGVRQCLGRRIAELEMTIFLINMLENFRVEIQHLSDVGTTFNLILMPEKPISFTFWPFNQEATQQ
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion membrane.
Function
- Function:
- Catalyzes the side-chain cleavage reaction of cholesterol to pregnenolone.
- Pathway:
- Lipid metabolism; C21-steroid hormone metabolism.
- Catalytic Activity:
- Cholesterol + 6 reduced adrenodoxin + 3 O(2) + 6 H(+) = pregnenolone + 4-methylpentanal + 6 oxidized adrenodoxin + 4 H(2)O.
- Cofactor:
- COFACTOR: Name=heme; Xref=ChEBI:CHEBI:30413;
- Cross Reference Drug Bank:
- DB00357 DB00169 DB00882 DB00257 DB01234 DB01396 DB00390 DB00917 DB01437 DB01026 DB00338 DB01232 DB00857 DB00624
- Gene Ontology Go:
- mitochondrial inner membrane
mitochondrial matrix
mitochondrion
cholesterol monooxygenase (side-chain-cleaving) activity
heme binding
iron ion binding
oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NAD(P)H as one donor, and incorporation of one atom of oxygen
C21-steroid hormone biosynthetic process
cellular response to peptide hormone stimulus
cholesterol metabolic process
cortisol metabolic process
glucocorticoid biosynthetic process
secondary metabolite biosynthetic process
small molecule metabolic process
steroid metabolic process
sterol metabolic process
vitamin D metabolic process
xenobiotic metabolic process - Gene Ontology Biological Process:
- C21-steroid hormone biosynthetic process
cellular response to peptide hormone stimulus
cholesterol metabolic process
cortisol metabolic process
glucocorticoid biosynthetic process
secondary metabolite biosynthetic process
small molecule metabolic process
steroid metabolic process
sterol metabolic process
vitamin D metabolic process
xenobiotic metabolic process - Gene Ontology Molecular Function:
- cholesterol monooxygenase (side-chain-cleaving) activity
heme binding
iron ion binding
oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NAD(P)H as one donor, and incorporation of one atom of oxygen - Gene Ontology Cellular Component:
- mitochondrial inner membrane
mitochondrial matrix
mitochondrion - Keywords:
- 3D-structure
Alternative splicing
Cholesterol metabolism
Complete proteome
Direct protein sequencing
Disease mutation
Heme
Iron
Lipid metabolism
Membrane
Metal-binding
Mitochondrion
Monooxygenase
Oxidoreductase
Polymorphism
Reference proteome
Steroid metabolism
Steroidogenesis
Sterol metabolism
Transit peptide