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Protein

Cholesterol side-chain cleavage enzyme, mitochondrial

Gene

Cyp11a1

Organism
Rattus norvegicus (Rat)
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 bindingi459 – 4591Iron (heme axial ligand)By similarity

GO - Molecular functioni

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

GO - Biological processi

  1. biphenyl metabolic process Source: RGD
  2. C21-steroid hormone biosynthetic process Source: RGD
  3. cellular response to antibiotic Source: RGD
  4. cellular response to cadmium ion Source: RGD
  5. cellular response to cAMP Source: RGD
  6. cellular response to fibroblast growth factor stimulus Source: RGD
  7. cellular response to follicle-stimulating hormone stimulus Source: RGD
  8. cellular response to gonadotropin stimulus Source: RGD
  9. cellular response to interleukin-1 Source: RGD
  10. cellular response to lipopolysaccharide Source: RGD
  11. cellular response to peptide hormone stimulus Source: RGD
  12. cellular response to transforming growth factor beta stimulus Source: RGD
  13. cellular response to tumor necrosis factor Source: RGD
  14. cerebellum development Source: RGD
  15. cholesterol metabolic process Source: UniProtKB
  16. dibenzo-p-dioxin metabolic process Source: RGD
  17. estrogen biosynthetic process Source: RGD
  18. granulosa cell differentiation Source: RGD
  19. hippocampus development Source: RGD
  20. Leydig cell differentiation Source: RGD
  21. male gonad development Source: RGD
  22. maternal process involved in female pregnancy Source: RGD
  23. mating behavior Source: RGD
  24. organic acid metabolic process Source: RGD
  25. phenol-containing compound metabolic process Source: RGD
  26. phthalate metabolic process Source: RGD
  27. progesterone biosynthetic process Source: RGD
  28. response to alkaloid Source: RGD
  29. response to amino acid Source: RGD
  30. response to antibiotic Source: RGD
  31. response to cadmium ion Source: RGD
  32. response to cAMP Source: RGD
  33. response to corticosterone Source: RGD
  34. response to drug Source: RGD
  35. response to estrogen Source: RGD
  36. response to fungicide Source: RGD
  37. response to gamma radiation Source: RGD
  38. response to genistein Source: RGD
  39. response to gonadotropin Source: RGD
  40. response to hydrogen peroxide Source: RGD
  41. response to insecticide Source: RGD
  42. response to ionizing radiation Source: RGD
  43. response to L-ascorbic acid Source: RGD
  44. response to nutrient Source: RGD
  45. response to organic cyclic compound Source: RGD
  46. response to organic substance Source: RGD
  47. response to peptide hormone Source: RGD
  48. response to salt stress Source: RGD
  49. response to steroid hormone Source: RGD
  50. response to vitamin E Source: RGD
  51. Schwann cell differentiation Source: RGD
  52. steroid biosynthetic process Source: RGD
  53. testosterone biosynthetic process Source: RGD
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_306856. Defective CYP11A1 causes Adrenal insufficiency, congenital, with 46,XY sex reversal (AICSR).
REACT_342548. Pregnenolone biosynthesis.
REACT_350068. Endogenous sterols.
SABIO-RKP14137.
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, Cyp11a-1
OrganismiRattus norvegicus (Rat)
Taxonomic identifieri10116 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeRattus
ProteomesiUP000002494 Componenti: Chromosome 8

Organism-specific databases

RGDi69325. Cyp11a1.

Subcellular locationi

GO - Cellular componenti

  1. mitochondrial crista Source: RGD
  2. mitochondrial inner membrane Source: RGD
  3. mitochondrion Source: RGD
  4. perikaryon Source: RGD
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3636MitochondrionAdd
BLAST
Chaini37 – 526490Cholesterol side-chain cleavage enzyme, mitochondrialPRO_0000003590Add
BLAST

Proteomic databases

PRIDEiP14137.

Expressioni

Tissue specificityi

Expressed in ovary, adrenal and testis.1 Publication

Inductioni

Induced by FSH or pregnant mare's serum gonadotropin in ovaries of estrogen-treated immature rats in vivo.

Gene expression databases

ExpressionAtlasiP14137. baseline and differential.
GenevestigatoriP14137.

Interactioni

Subunit structurei

Interacts with FDX1/adrenodoxin.By similarity

Structurei

3D structure databases

ProteinModelPortaliP14137.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the cytochrome P450 family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiCOG2124.
GeneTreeiENSGT00760000119243.
HOGENOMiHOG000013161.
HOVERGENiHBG051098.
InParanoidiP14137.
KOiK00498.
OMAiDLIHFRN.
OrthoDBiEOG7MKW5S.
PhylomeDBiP14137.

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.

P14137-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MLAKGLCLRS VLVKSCQPFL SPVWQGPGLA TGNGAGISST NSPRSFNEIP
60 70 80 90 100
SPGDNGWINL YHFLRENGTH RIHYHHMQNF QKYGPIYREK LGNMESVYIL
110 120 130 140 150
DPKDAATLFS CEGPNPERYL VPPWVAYHQY YQRPIGVLFK SSDAWRKDRI
160 170 180 190 200
VLNQEVMAPD SIKNFVPLLE GVAQDFIKVL HRRIKQQNSG KFSGDISDDL
210 220 230 240 250
FRFAFESITS VVFGERLGML EEIVDPESQR FIDAVYQMFH TSVPMLNMPP
260 270 280 290 300
DLFRLFRTKT WKDHAAAWDV IFSKADEYTQ NFYWDLRQKR DFSKYPGVLY
310 320 330 340 350
SLLGGNKLPF KNIQANITEM LAGGVDTTSM TLQWNLYEMA HNLKVQEMLR
360 370 380 390 400
AEVLAARRQA QGDMAKMVQL VPLLKASIKE TLRLHPISVT LQRYIVNDLV
410 420 430 440 450
LRNYKIPAKT LVQVASYAMG RESSFFPNPN KFDPTRWLEK SQNTTHFRYL
460 470 480 490 500
GFGWGVRQCL GRRIAELEMT IFLINVLENF RIEVQSIRDV GTKFNLILMP
510 520
EKPIFFNFQP LKQDLGSTMP RKGDTV
Length:526
Mass (Da):60,586
Last modified:January 1, 1990 - v1
Checksum:iB2930E40018DC1EE
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J05156 mRNA. Translation: AAA40989.1.
M63133
, M63125, M63126, M63127, M63128, M63129, M63130, M63131, M63132 Genomic DNA. Translation: AAA40958.1.
BC089100 mRNA. Translation: AAH89100.1.
M22615 mRNA. Translation: AAA62267.1.
PIRiA34164.
RefSeqiNP_058982.1. NM_017286.2.
UniGeneiRn.1401.

Genome annotation databases

EnsembliENSRNOT00000010831; ENSRNOP00000010831; ENSRNOG00000008074.
GeneIDi29680.
KEGGirno:29680.
UCSCiRGD:69325. rat.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J05156 mRNA. Translation: AAA40989.1.
M63133
, M63125, M63126, M63127, M63128, M63129, M63130, M63131, M63132 Genomic DNA. Translation: AAA40958.1.
BC089100 mRNA. Translation: AAH89100.1.
M22615 mRNA. Translation: AAA62267.1.
PIRiA34164.
RefSeqiNP_058982.1. NM_017286.2.
UniGeneiRn.1401.

3D structure databases

ProteinModelPortaliP14137.
ModBaseiSearch...
MobiDBiSearch...

Chemistry

BindingDBiP14137.
ChEMBLiCHEMBL5246.

Proteomic databases

PRIDEiP14137.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSRNOT00000010831; ENSRNOP00000010831; ENSRNOG00000008074.
GeneIDi29680.
KEGGirno:29680.
UCSCiRGD:69325. rat.

Organism-specific databases

CTDi1583.
RGDi69325. Cyp11a1.

Phylogenomic databases

eggNOGiCOG2124.
GeneTreeiENSGT00760000119243.
HOGENOMiHOG000013161.
HOVERGENiHBG051098.
InParanoidiP14137.
KOiK00498.
OMAiDLIHFRN.
OrthoDBiEOG7MKW5S.
PhylomeDBiP14137.

Enzyme and pathway databases

UniPathwayiUPA00229.
ReactomeiREACT_306856. Defective CYP11A1 causes Adrenal insufficiency, congenital, with 46,XY sex reversal (AICSR).
REACT_342548. Pregnenolone biosynthesis.
REACT_350068. Endogenous sterols.
SABIO-RKP14137.

Miscellaneous databases

NextBioi610031.
PROiP14137.

Gene expression databases

ExpressionAtlasiP14137. baseline and differential.
GenevestigatoriP14137.

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

« Hide 'large scale' publications
  1. "Cyclic AMP-dependent and -independent regulation of cholesterol side chain cleavage cytochrome P-450 (P-450scc) in rat ovarian granulosa cells and corpora lutea. cDNA and deduced amino acid sequence of rat P-450scc."
    Oonk R.B., Krasnow J.S., Beattie W.G., Richards J.S.
    J. Biol. Chem. 264:21934-21942(1989) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
  2. "Rat cholesterol side-chain cleavage cytochrome P-450 (P-450scc) gene. Structure and regulation by cAMP in vitro."
    Oonk R.B., Parker K.L., Gibson J.L., Richards J.S.
    J. Biol. Chem. 265:22392-22401(1990) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
  3. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Ovary.
  4. "Rat cholesterol side-chain cleavage enzyme (P-450scc): use of a cDNA probe to study the hormonal regulation of P-450scc mRNA levels in ovarian granulosa cells."
    McMasters K.M., Dickson L.A., Shamy R.V., Robischon K., Macdonald G.J., Moyle W.R.
    Gene 57:1-9(1987) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 344-526, TISSUE SPECIFICITY.
    Strain: Sprague-Dawley.
    Tissue: Ovary.

Entry informationi

Entry nameiCP11A_RAT
AccessioniPrimary (citable) accession number: P14137
Secondary accession number(s): Q5FWY8, Q6LDR9
Entry historyi
Integrated into UniProtKB/Swiss-Prot: January 1, 1990
Last sequence update: January 1, 1990
Last modified: April 1, 2015
This is version 125 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. 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.