Gene/Proteome Database (LMPD)

LMPD ID
LMP000259
Gene ID
1583
Species
Homo sapiens (Human)
Gene Name
cytochrome P450, family 11, subfamily A, polypeptide 1
Gene Symbol
CYP11A1
Synonyms
CYP11A; CYPXIA1; P450SCC
Alternate Names
cholesterol side-chain cleavage enzyme, mitochondrial; steroid 20-22-lyase; cytochrome P450 11A1; cytochrome P450(scc); cytochrome P450C11A1; cholesterol 20-22 desmolase; cholesterol monooxygenase (side-chain cleaving); cytochrome P450, subfamily XIA (cholesterol side chain cleavage)
Chromosome
15
Map Location
15q23-q24
EC Number
1.14.15.6
Summary
This gene encodes a member of the cytochrome P450 superfamily of enzymes. The cytochrome P450 proteins are monooxygenases which catalyze many reactions involved in drug metabolism and synthesis of cholesterol, steroids and other lipids. This protein localizes to the mitochondrial inner membrane and catalyzes the conversion of cholesterol to pregnenolone, the first and rate-limiting step in the synthesis of the steroid hormones. Two transcript variants encoding different isoforms have been found for this gene. The cellular location of the smaller isoform is unclear since it lacks the mitochondrial-targeting transit peptide. [provided by RefSeq, Jul 2008]
Orthologs
View orthologs and multiple alignments for CYP11A1

Proteins

cholesterol side-chain cleavage enzyme, mitochondrial isoform a precursor
Refseq ID NP_000772
Protein GI 153218646
UniProt ID P05108
mRNA ID NM_000781
Length 521
RefSeq Status REVIEWED
MLAKGLPPRSVLVKGCQTFLSAPREGLGRLRVPTGEGAGISTRSPRPFNEIPSPGDNGWLNLYHFWRETGTHKVHLHHVQNFQKYGPIYREKLGNVESVYVIDPEDVALLFKSEGPNPERFLIPPWVAYHQYYQRPIGVLLKKSAAWKKDRVALNQEVMAPEATKNFLPLLDAVSRDFVSVLHRRIKKAGSGNYSGDISDDLFRFAFESITNVIFGERQGMLEEVVNPEAQRFIDAIYQMFHTSVPMLNLPPDLFRLFRTKTWKDHVAAWDVIFSKADIYTQNFYWELRQKGSVHHDYRGILYRLLGDSKMSFEDIKANVTEMLAGGVDTTSMTLQWHLYEMARNLKVQDMLRAEVLAARHQAQGDMATMLQLVPLLKASIKETLRLHPISVTLQRYLVNDLVLRDYMIPAKTLVQVAIYALGREPTFFFDPENFDPTRWLSKDKNITYFRNLGFGWGVRQCLGRRIAELEMTIFLINMLENFRVEIQHLSDVGTTFNLILMPEKPISFTFWPFNQEATQQ
cholesterol side-chain cleavage enzyme, mitochondrial isoform b
Refseq ID NP_001093243
Protein GI 153218654
UniProt ID P05108
mRNA ID NM_001099773
Length 363
RefSeq Status REVIEWED
MAPEATKNFLPLLDAVSRDFVSVLHRRIKKAGSGNYSGDISDDLFRFAFESITNVIFGERQGMLEEVVNPEAQRFIDAIYQMFHTSVPMLNLPPDLFRLFRTKTWKDHVAAWDVIFSKADIYTQNFYWELRQKGSVHHDYRGILYRLLGDSKMSFEDIKANVTEMLAGGVDTTSMTLQWHLYEMARNLKVQDMLRAEVLAARHQAQGDMATMLQLVPLLKASIKETLRLHPISVTLQRYLVNDLVLRDYMIPAKTLVQVAIYALGREPTFFFDPENFDPTRWLSKDKNITYFRNLGFGWGVRQCLGRRIAELEMTIFLINMLENFRVEIQHLSDVGTTFNLILMPEKPISFTFWPFNQEATQQ
transit_peptide: 1..39 calculated_mol_wt: 4021 peptide sequence: MLAKGLPPRSVLVKGCQTFLSAPREGLGRLRVPTGEGAG mat_peptide: 40..521 product: cholesterol side-chain cleavage enzyme, mitochondrial isoform a calculated_mol_wt: 56100 peptide sequence: ISTRSPRPFNEIPSPGDNGWLNLYHFWRETGTHKVHLHHVQNFQKYGPIYREKLGNVESVYVIDPEDVALLFKSEGPNPERFLIPPWVAYHQYYQRPIGVLLKKSAAWKKDRVALNQEVMAPEATKNFLPLLDAVSRDFVSVLHRRIKKAGSGNYSGDISDDLFRFAFESITNVIFGERQGMLEEVVNPEAQRFIDAIYQMFHTSVPMLNLPPDLFRLFRTKTWKDHVAAWDVIFSKADIYTQNFYWELRQKGSVHHDYRGILYRLLGDSKMSFEDIKANVTEMLAGGVDTTSMTLQWHLYEMARNLKVQDMLRAEVLAARHQAQGDMATMLQLVPLLKASIKETLRLHPISVTLQRYLVNDLVLRDYMIPAKTLVQVAIYALGREPTFFFDPENFDPTRWLSKDKNITYFRNLGFGWGVRQCLGRRIAELEMTIFLINMLENFRVEIQHLSDVGTTFNLILMPEKPISFTFWPFNQEATQQ

Gene Information

Entrez Gene ID
1583
Gene Name
cytochrome P450, family 11, subfamily A, polypeptide 1
Gene Symbol
CYP11A1
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0030061 IEA:Ensembl C mitochondrial crista
GO:0005759 TAS:Reactome C mitochondrial matrix
GO:0005739 ISS:UniProtKB C mitochondrion
GO:0043204 IEA:Ensembl C perikaryon
GO:0015485 IEA:Ensembl F cholesterol binding
GO:0008386 IDA:UniProtKB F cholesterol monooxygenase (side-chain-cleaving) activity
GO:0020037 IDA:UniProtKB F heme binding
GO:0005506 IEA:InterPro F iron ion binding
GO:0006700 IDA:UniProtKB P C21-steroid hormone biosynthetic process
GO:0033327 IEA:Ensembl P Leydig cell differentiation
GO:0014037 IEA:Ensembl P Schwann cell differentiation
GO:0018879 IEA:Ensembl P biphenyl metabolic process
GO:0071236 IEA:Ensembl P cellular response to antibiotic
GO:0071320 IEA:Ensembl P cellular response to cAMP
GO:0071276 IEA:Ensembl P cellular response to cadmium ion
GO:0044344 IEA:Ensembl P cellular response to fibroblast growth factor stimulus
GO:0071372 IEA:Ensembl P cellular response to follicle-stimulating hormone stimulus
GO:0071347 IEA:Ensembl P cellular response to interleukin-1
GO:0071222 IEA:Ensembl P cellular response to lipopolysaccharide
GO:0071375 IEA:Ensembl P cellular response to peptide hormone stimulus
GO:0071560 IEA:Ensembl P cellular response to transforming growth factor beta stimulus
GO:0071356 IEA:Ensembl P cellular response to tumor necrosis factor
GO:0021549 IEA:Ensembl P cerebellum development
GO:0008203 IDA:UniProtKB P cholesterol metabolic process
GO:0018894 IEA:Ensembl P dibenzo-p-dioxin metabolic process
GO:0006703 IEA:Ensembl P estrogen biosynthetic process
GO:0050756 IEA:Ensembl P fractalkine metabolic process
GO:0060014 IEA:Ensembl P granulosa cell differentiation
GO:0021766 IEA:Ensembl P hippocampus development
GO:0060135 IEA:Ensembl P maternal process involved in female pregnancy
GO:0007617 IEA:Ensembl P mating behavior
GO:0018958 IEA:Ensembl P phenol-containing compound metabolic process
GO:0018963 IEA:Ensembl P phthalate metabolic process
GO:0006701 IEA:Ensembl P progesterone biosynthetic process
GO:0033591 IEA:Ensembl P response to L-ascorbic acid
GO:0043279 IEA:Ensembl P response to alkaloid
GO:0051412 IEA:Ensembl P response to corticosterone
GO:0042493 IEA:Ensembl P response to drug
GO:0060992 IEA:Ensembl P response to fungicide
GO:0010332 IEA:Ensembl P response to gamma radiation
GO:0033595 IEA:Ensembl P response to genistein
GO:0042542 IEA:Ensembl P response to hydrogen peroxide
GO:0017085 IEA:Ensembl P response to insecticide
GO:0009651 IEA:Ensembl P response to salt stress
GO:0033197 IEA:Ensembl P response to vitamin E
GO:0044281 TAS:Reactome P small molecule metabolic process
GO:0008202 TAS:Reactome P steroid metabolic process
GO:0016125 TAS:Reactome P sterol metabolic process
GO:0061370 IEA:Ensembl P testosterone biosynthetic process
GO:0042359 ISS:UniProtKB P vitamin D metabolic process
GO:0006805 TAS:Reactome P xenobiotic metabolic process

KEGG Pathway Links

KEGG Pathway ID Description
hsa04913 Ovarian steroidogenesis
hsa00140 Steroid hormone biosynthesis

Domain Information

InterPro Annotations

Accession Description
IPR001128 Cytochrome P450
IPR002401 Cytochrome P450, E-class, group I
IPR017972 Cytochrome P450, conserved site

UniProt Annotations

Entry Information

Gene Name
cytochrome P450, family 11, subfamily A, polypeptide 1
Protein Entry
CP11A_HUMAN
UniProt ID
P05108
Species
Human

Comments

Comment Type Description
Alternative Products Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=P05108-1; Sequence=Displayed; Name=2; IsoId=P05108-2; Sequence=VSP_045695; Note=No experimental confirmation available.;
Catalytic Activity Cholesterol + 6 reduced adrenodoxin + 3 O(2) + 6 H(+) = pregnenolone + 4-methylpentanal + 6 oxidized adrenodoxin + 4 H(2)O.
Cofactor Name=heme; Xref=ChEBI
Disease Adrenal insufficiency, congenital, with 46,XY sex reversal (AICSR) [MIM
Function Catalyzes the side-chain cleavage reaction of cholesterol to pregnenolone.
Induction By 8-bromo cyclic AMP.
Pathway Lipid metabolism; C21-steroid hormone metabolism.
Similarity Belongs to the cytochrome P450 family.
Subcellular Location Mitochondrion membrane.
Subunit Interacts with FDX1/adrenodoxin.

Identical and Related Proteins

Unique RefSeq proteins for LMP000259 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
153218646 RefSeq NP_000772 521 cholesterol side-chain cleavage enzyme, mitochondrial isoform a precursor
153218654 RefSeq NP_001093243 363 cholesterol side-chain cleavage enzyme, mitochondrial isoform b

Identical Sequences to LMP000259 proteins

Reference Database Accession Length Protein Name
GI:153218646 DBBJ BAF84989.1 521 unnamed protein product [Homo sapiens]
GI:153218646 DBBJ BAI46612.1 521 cytochrome P450, family 11, subfamily A, polypeptide 1, partial [synthetic construct]
GI:153218654 GenBank EAW99341.1 363 cytochrome P450, family 11, subfamily A, polypeptide 1, isoform CRA_a [Homo sapiens]
GI:153218646 GenBank EAW99342.1 521 cytochrome P450, family 11, subfamily A, polypeptide 1, isoform CRA_b [Homo sapiens]
GI:153218646 GenBank ABM82504.1 521 cytochrome P450, family 11, subfamily A, polypeptide 1 [synthetic construct]
GI:153218646 GenBank ABM85697.1 521 cytochrome P450, family 11, subfamily A, polypeptide 1, partial [synthetic construct]
GI:153218646 SwissProt P05108.2 521 RecName: Full=Cholesterol side-chain cleavage enzyme, mitochondrial; AltName: Full=CYPXIA1; AltName: Full=Cholesterol desmolase; AltName: Full=Cytochrome P450 11A1; AltName: Full=Cytochrome P450(scc); Flags: Precursor [Homo sapiens]

Related Sequences to LMP000259 proteins

Reference Database Accession Length Protein Name
GI:153218654 DBBJ BAF84989.1 521 unnamed protein product [Homo sapiens]
GI:153218654 DBBJ BAI46612.1 521 cytochrome P450, family 11, subfamily A, polypeptide 1, partial [synthetic construct]
GI:153218646 DBBJ BAK61896.1 521 cytochrome P450 11A1, mitochondrial precursor [Pan troglodytes]
GI:153218646 EMBL CAA28965.1 521 desmolase [Homo sapiens]
GI:153218646 GenBank AAA52162.1 521 cholesterol side-chain cleavage enzyme P450scc (EC 1.14.15.67) [Homo sapiens]
GI:153218654 GenBank AAH32329.1 521 Cytochrome P450, family 11, subfamily A, polypeptide 1 [Homo sapiens]
GI:153218654 GenBank AAX42484.1 521 cytochrome P450 family 11 subfamily A polypeptide 1 [synthetic construct]
GI:153218646 GenBank AIC54258.1 521 CYP11A1, partial [synthetic construct]
GI:153218646 PRF - 521 cytochrome P450 [Homo sapiens]
GI:153218654 RefSeq NP_000772.2 521 cholesterol side-chain cleavage enzyme, mitochondrial isoform a precursor [Homo sapiens]
GI:153218646 RefSeq NP_001267337.1 521 cholesterol side-chain cleavage enzyme, mitochondrial [Pan troglodytes]
GI:153218654 SwissProt P05108.2 521 RecName: Full=Cholesterol side-chain cleavage enzyme, mitochondrial; AltName: Full=CYPXIA1; AltName: Full=Cholesterol desmolase; AltName: Full=Cytochrome P450 11A1; AltName: Full=Cytochrome P450(scc); Flags: Precursor [Homo sapiens]