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Protein

Cholesterol side-chain cleavage enzyme, mitochondrial

Gene

CYP11A1

Organism
Sus scrofa (Pig)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-Experimental evidence at protein leveli

Functioni

Catalyzes the side-chain cleavage reaction of cholesterol to pregnenolone.By similarity

Catalytic activityi

Cholesterol + 6 reduced adrenodoxin + 3 O2 + 6 H+ = pregnenolone + 4-methylpentanal + 6 oxidized adrenodoxin + 4 H2O.By similarity

Cofactori

hemeBy similarity

Pathwayi

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi461 – 4611Iron (heme axial ligand)

GO - Molecular functioni

  1. cholesterol monooxygenase (side-chain-cleaving) activity Source: UniProtKB
  2. heme binding Source: UniProtKB
  3. iron ion binding Source: InterPro

GO - Biological processi

  1. C21-steroid hormone biosynthetic process Source: UniProtKB
  2. cholesterol metabolic process Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Monooxygenase, Oxidoreductase

Keywords - Biological processi

Cholesterol metabolism, Lipid metabolism, Steroid metabolism, Steroidogenesis, Sterol metabolism

Keywords - Ligandi

Heme, Iron, Metal-binding

Enzyme and pathway databases

ReactomeiREACT_300563. Defective CYP11A1 causes Adrenal insufficiency, congenital, with 46,XY sex reversal (AICSR).
REACT_307884. Endogenous sterols.
REACT_347635. Pregnenolone biosynthesis.
UniPathwayiUPA00229.

Names & Taxonomyi

Protein namesi
Recommended name:
Cholesterol side-chain cleavage enzyme, mitochondrial (EC:1.14.15.6)
Alternative name(s):
CYPXIA1
Cholesterol desmolase
Cytochrome P450 11A1
Cytochrome P450(scc)
Gene namesi
Name:CYP11A1
OrganismiSus scrofa (Pig)
Taxonomic identifieri9823 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaLaurasiatheriaCetartiodactylaSuinaSuidaeSus
ProteomesiUP000008227 Componenti: Unplaced

Subcellular locationi

GO - Cellular componenti

  1. mitochondrial membrane Source: UniProtKB-SubCell
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3939Mitochondrion1 PublicationAdd
BLAST
Chaini40 – 520481Cholesterol side-chain cleavage enzyme, mitochondrialPRO_0000003588Add
BLAST

Interactioni

Subunit structurei

Interacts with FDX1/adrenodoxin.By similarity

Structurei

3D structure databases

ProteinModelPortaliP10612.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the cytochrome P450 family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

HOVERGENiHBG051098.
InParanoidiP10612.
KOiK00498.

Family and domain databases

Gene3Di1.10.630.10. 1 hit.
InterProiIPR001128. Cyt_P450.
IPR017972. Cyt_P450_CS.
IPR002401. Cyt_P450_E_grp-I.
[Graphical view]
PfamiPF00067. p450. 1 hit.
[Graphical view]
PRINTSiPR00463. EP450I.
PR00385. P450.
SUPFAMiSSF48264. SSF48264. 1 hit.
PROSITEiPS00086. CYTOCHROME_P450. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

P10612-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MLARGLALRS VLVKGCQPFL SAPRECPGHP RVGTGEGACI STKTPRPFSE
60 70 80 90 100
IPSPGDNGWI NLYRFWKEKG TQKIHYHHVQ NFQKYGPIYR EKLGNLESVY
110 120 130 140 150
IIDPEDVALL FKFEGPNPER YNIPPWVAYH QHYQKPVGVL LKKSGAWKKD
160 170 180 190 200
RLVLNTEVMA PEAIKNFIPL LDTVSQDFVG VLHRRIKQQG SGKFSGDIRE
210 220 230 240 250
DLFRFAFESI TNVIFGERLG MLEEIVDPEA QKFIDAVYQM FHTSVPMLNL
260 270 280 290 300
PPDLFRLFRT KTWRDHVAAW DTIFNKAEKY TQNFYWDLRR KREFNNYPGI
310 320 330 340 350
LYRLLGNDKL LSEDVKANVT EMLAGGVDTT SMTLQWHLYE MARSLNVQEM
360 370 380 390 400
LREEVLNARR QAQGDTSKML QLVPLLKASI KETLRLHPIS VTLQRYLVND
410 420 430 440 450
LVLRDYMIPA KTLVQVAVYA MGRDPAFFSN PGQFDPTRWL GKERDLIHFR
460 470 480 490 500
NLGFGWGVRQ CVGRRIAELE MTLFLIHILE NFKVELQHFS DVDTIFNLIL
510 520
MPDKPIFLVF RPFNQDPLQA
Length:520
Mass (Da):60,258
Last modified:June 30, 1989 - v1
Checksum:i6C6D0D6FE34F9C33
GO

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti46 – 461R → F AA sequence (PubMed:1773895).Curated

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X13768 mRNA. Translation: CAA32018.1.
PIRiS03188.
RefSeqiNP_999592.1. NM_214427.1.
UniGeneiSsc.8776.

Genome annotation databases

GeneIDi403329.
KEGGissc:403329.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X13768 mRNA. Translation: CAA32018.1.
PIRiS03188.
RefSeqiNP_999592.1. NM_214427.1.
UniGeneiSsc.8776.

3D structure databases

ProteinModelPortaliP10612.
ModBaseiSearch...
MobiDBiSearch...

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

GeneIDi403329.
KEGGissc:403329.

Organism-specific databases

CTDi1583.

Phylogenomic databases

HOVERGENiHBG051098.
InParanoidiP10612.
KOiK00498.

Enzyme and pathway databases

UniPathwayiUPA00229.
ReactomeiREACT_300563. Defective CYP11A1 causes Adrenal insufficiency, congenital, with 46,XY sex reversal (AICSR).
REACT_307884. Endogenous sterols.
REACT_347635. Pregnenolone biosynthesis.

Family and domain databases

Gene3Di1.10.630.10. 1 hit.
InterProiIPR001128. Cyt_P450.
IPR017972. Cyt_P450_CS.
IPR002401. Cyt_P450_E_grp-I.
[Graphical view]
PfamiPF00067. p450. 1 hit.
[Graphical view]
PRINTSiPR00463. EP450I.
PR00385. P450.
SUPFAMiSSF48264. SSF48264. 1 hit.
PROSITEiPS00086. CYTOCHROME_P450. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

  1. "Nucleotide sequence of cytochrome P-450 cholesterol side-chain cleavage cDNA isolated from porcine testis."
    Mulheron G.W., Stone R.T., Miller W.L., Wise T.H.
    Nucleic Acids Res. 17:1773-1773(1988) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    Tissue: Testis.
  2. "Purification and properties of cytochrome P-450 (SCC) from pig testis mitochondria."
    Kuwada M., Kitajima R., Suzuki H., Horie S.
    Biochem. Biophys. Res. Commun. 176:1501-1508(1990) [PubMed] [Europe PMC] [Abstract]
    Cited for: PROTEIN SEQUENCE OF 41-61.
    Tissue: Testis.
  3. "Purification and comparative characterization of cytochrome P-450scc from porcine adrenocortical mitochondria."
    Iwahashi K., Tsubaki M., Miyatake A., Ichikawa Y.
    Int. J. Biochem. 23:901-909(1990) [PubMed] [Europe PMC] [Abstract]
    Cited for: PROTEIN SEQUENCE OF 40-64.

Entry informationi

Entry nameiCP11A_PIG
AccessioniPrimary (citable) accession number: P10612
Entry historyi
Integrated into UniProtKB/Swiss-Prot: June 30, 1989
Last sequence update: June 30, 1989
Last modified: March 31, 2015
This is version 103 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  2. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.