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Protein

Cholesterol side-chain cleavage enzyme, mitochondrial

Gene

Cyp11a1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at transcript 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 bindingi458 – 4581Iron (heme axial ligand)By similarity

GO - Molecular functioni

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

GO - Biological processi

  1. biphenyl metabolic process Source: Ensembl
  2. C21-steroid hormone biosynthetic process Source: MGI
  3. cellular response to antibiotic Source: Ensembl
  4. cellular response to cadmium ion Source: Ensembl
  5. cellular response to cAMP Source: Ensembl
  6. cellular response to fibroblast growth factor stimulus Source: Ensembl
  7. cellular response to follicle-stimulating hormone stimulus Source: Ensembl
  8. cellular response to interleukin-1 Source: Ensembl
  9. cellular response to lipopolysaccharide Source: Ensembl
  10. cellular response to peptide hormone stimulus Source: Ensembl
  11. cellular response to transforming growth factor beta stimulus Source: Ensembl
  12. cellular response to tumor necrosis factor Source: Ensembl
  13. cerebellum development Source: Ensembl
  14. cholesterol metabolic process Source: UniProtKB
  15. dibenzo-p-dioxin metabolic process Source: Ensembl
  16. estrogen biosynthetic process Source: Ensembl
  17. fractalkine metabolic process Source: Ensembl
  18. granulosa cell differentiation Source: Ensembl
  19. hippocampus development Source: Ensembl
  20. Leydig cell differentiation Source: Ensembl
  21. maternal process involved in female pregnancy Source: Ensembl
  22. mating behavior Source: Ensembl
  23. phenol-containing compound metabolic process Source: Ensembl
  24. phthalate metabolic process Source: Ensembl
  25. progesterone biosynthetic process Source: Ensembl
  26. response to alkaloid Source: Ensembl
  27. response to corticosterone Source: Ensembl
  28. response to drug Source: Ensembl
  29. response to fungicide Source: Ensembl
  30. response to gamma radiation Source: Ensembl
  31. response to genistein Source: Ensembl
  32. response to hydrogen peroxide Source: Ensembl
  33. response to insecticide Source: Ensembl
  34. response to L-ascorbic acid Source: Ensembl
  35. response to salt stress Source: Ensembl
  36. response to vitamin E Source: Ensembl
  37. Schwann cell differentiation Source: Ensembl
  38. testosterone biosynthetic process Source: Ensembl
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_281686. Endogenous sterols.
REACT_294407. Pregnenolone biosynthesis.
REACT_332536. Defective CYP11A1 causes Adrenal insufficiency, congenital, with 46,XY sex reversal (AICSR).
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
Synonyms:Cyp11a
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
ProteomesiUP000000589 Componenti: Chromosome 9

Organism-specific databases

MGIiMGI:88582. Cyp11a1.

Subcellular locationi

GO - Cellular componenti

  1. mitochondrial crista Source: Ensembl
  2. mitochondrion Source: MGI
  3. perikaryon Source: Ensembl
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3636MitochondrionBy similarityAdd
BLAST
Chaini37 – 526490Cholesterol side-chain cleavage enzyme, mitochondrialPRO_0000003587Add
BLAST

Proteomic databases

MaxQBiQ9QZ82.
PaxDbiQ9QZ82.
PRIDEiQ9QZ82.

PTM databases

PhosphoSiteiQ9QZ82.

Expressioni

Gene expression databases

BgeeiQ9QZ82.
GenevestigatoriQ9QZ82.

Interactioni

Subunit structurei

Interacts with FDX1/adrenodoxin.By similarity

Structurei

3D structure databases

ProteinModelPortaliQ9QZ82.
SMRiQ9QZ82. Positions 41-510.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the cytochrome P450 family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiCOG2124.
GeneTreeiENSGT00760000119243.
HOGENOMiHOG000013161.
HOVERGENiHBG051098.
InParanoidiQ9QZ82.
KOiK00498.
OMAiDLIHFRN.
OrthoDBiEOG7MKW5S.
PhylomeDBiQ9QZ82.
TreeFamiTF105094.

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.

Q9QZ82-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MLAKGLSLRS VLVKGCQPFL SPTWQGPVLS TGKGAGTSTS SPRSFNEIPS
60 70 80 90 100
PGDNGWLNLY HFWRESGTQK IHYHQMQSFQ KYGPIYREKL GTLESVYIVD
110 120 130 140 150
PKDASILFSC EGPNPERFLV PPWVAYHQYY QRPIGVLFKS SDAWKKDRIV
160 170 180 190 200
LNQEVMAPGA IKNFVPLLEG VAQDFIKVLH RRIKQQNSGN FSGVISDDLF
210 220 230 240 250
RFSFESISSV IFGERMGMLE EIVDPEAQRF INAVYQMFHT SVPMLNLPPD
260 270 280 290 300
FFRLLRTKTW KDHAAAWDVI FNKADEYTQN FYWDLRQKRD FSQYPGVLYS
310 320 330 340 350
LLGGNKLPFK NIQANITEML AGGVDTTSMT LQWNLYEMAH NLKVQEMLRA
360 370 380 390 400
EVLAARRQAQ GDMAKMVQLV PLLKASIKET LRLHPISVTL QRYTVNDLVL
410 420 430 440 450
RNYKIPAKTL VQVASFAMGR DPGFFPNPNK FDPTRWLEKS QNTTHFRYLG
460 470 480 490 500
FGWGVRQCLG RRIAELEMTI LLINLLENFR IEVQNLRDVG TKFSLILMPE
510 520
NPILFNFQPL KQDLGPAVTR KDNTVN
Length:526
Mass (Da):60,315
Last modified:April 30, 2000 - v1
Checksum:iE5029738EE4ECB71
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF195119 mRNA. Translation: AAF03897.1.
CCDSiCCDS40653.1.
RefSeqiNP_062753.3. NM_019779.3.
UniGeneiMm.302865.

Genome annotation databases

EnsembliENSMUST00000034874; ENSMUSP00000034874; ENSMUSG00000032323.
GeneIDi13070.
KEGGimmu:13070.
UCSCiuc009pwa.1. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF195119 mRNA. Translation: AAF03897.1.
CCDSiCCDS40653.1.
RefSeqiNP_062753.3. NM_019779.3.
UniGeneiMm.302865.

3D structure databases

ProteinModelPortaliQ9QZ82.
SMRiQ9QZ82. Positions 41-510.
ModBaseiSearch...
MobiDBiSearch...

PTM databases

PhosphoSiteiQ9QZ82.

Proteomic databases

MaxQBiQ9QZ82.
PaxDbiQ9QZ82.
PRIDEiQ9QZ82.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000034874; ENSMUSP00000034874; ENSMUSG00000032323.
GeneIDi13070.
KEGGimmu:13070.
UCSCiuc009pwa.1. mouse.

Organism-specific databases

CTDi1583.
MGIiMGI:88582. Cyp11a1.

Phylogenomic databases

eggNOGiCOG2124.
GeneTreeiENSGT00760000119243.
HOGENOMiHOG000013161.
HOVERGENiHBG051098.
InParanoidiQ9QZ82.
KOiK00498.
OMAiDLIHFRN.
OrthoDBiEOG7MKW5S.
PhylomeDBiQ9QZ82.
TreeFamiTF105094.

Enzyme and pathway databases

UniPathwayiUPA00229.
ReactomeiREACT_281686. Endogenous sterols.
REACT_294407. Pregnenolone biosynthesis.
REACT_332536. Defective CYP11A1 causes Adrenal insufficiency, congenital, with 46,XY sex reversal (AICSR).

Miscellaneous databases

NextBioi283004.
PROiQ9QZ82.
SOURCEiSearch...

Gene expression databases

BgeeiQ9QZ82.
GenevestigatoriQ9QZ82.

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. Tanaka M., Hennebold J.H., Adashi E.Y.
    Submitted (SEP-1999) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    Strain: C57BL/6.

Entry informationi

Entry nameiCP11A_MOUSE
AccessioniPrimary (citable) accession number: Q9QZ82
Entry historyi
Integrated into UniProtKB/Swiss-Prot: May 3, 2001
Last sequence update: April 30, 2000
Last modified: March 31, 2015
This is version 115 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, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. 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.