Reviewed, UniProtKB/Swiss-Prot Q28827 (CP11A_RABIT)

Names and origin

Protein names

Recommended name:
    Cholesterol side-chain cleavage enzyme, mitochondrial
    EC=1.14.15.6
Alternative name(s):
    Cytochrome P450 11A1
    CYPXIA1
    P450(scc)
    Cholesterol desmolase

Gene names

Name:

CYP11A1

Organism

Oryctolagus cuniculus (Rabbit)

Taxonomic identifier

9986 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Lagomorpha › Leporidae › Oryctolagus

Protein attributes

Sequence length	445 AA.
Sequence status	Fragment.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at transcript level.

General annotation (Comments)

Function	Catalyzes the side-chain cleavage reaction of cholesterol to pregnenolone.
Catalytic activity	Cholesterol + reduced adrenal ferredoxin + O2 = pregnenolone + 4-methylpentanal + oxidized adrenal ferredoxin + H2O.
Cofactor	Heme group By similarity.
Pathway	Lipid metabolism; C21-steroid hormone metabolism.
Subcellular location	Mitochondrion membrane.
Sequence similarities	Belongs to the cytochrome P450 family.

Ontologies

Keywords
Biological process	Cholesterol metabolism Lipid metabolism Steroid metabolism Steroidogenesis
Cellular component	Membrane Mitochondrion
Domain	Transit peptide
Ligand	Heme Iron Metal-binding
Molecular function	Monooxygenase Oxidoreductase
Gene Ontology (GO)
Biological process	cholesterol metabolic process Inferred from electronic annotation. Source: UniProtKB-KW oxidation reduction Inferred from electronic annotation. Source: UniProtKB-KW steroid biosynthetic process Inferred from electronic annotation. Source: UniProtKB-KW
Cellular component	mitochondrial membrane Inferred from electronic annotation. Source: UniProtKB-SubCell
Molecular function	cholesterol monooxygenase (side-chain-cleaving) activity Inferred from electronic annotation. Source: EC electron carrier activity Inferred from electronic annotation. Source: InterPro heme binding Inferred from electronic annotation. Source: InterPro
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	‹1 – 36	›36	Mitochondrion By similarity
	Chain	37 – ›445	›409	Cholesterol side-chain cleavage enzyme, mitochondrial		PRO_0000003589
Experimental info
	Non-terminal residue	1	1
	Non-terminal residue	445	1

Sequences

	Sequence		Length	Mass (Da)
	Q28827-1 [UniParc]. Last modified November 1, 1996. Version 1. Checksum: B6080DFFB028C8CA	FASTA	445	51,016
	10 20 30 40 50 60 RGLPSRSVFL RGCQASLSTA QERLGHPGVP TREGVRVATR SPRPYHEIPS PGDNGWLNLY 70 80 90 100 110 120 HLAEEKGTHR VHYRHVQNFQ KYGPIYRENL GNVESVYIMD PEDVALLFNS EGPQPERFLI 130 140 150 160 170 180 PPWVAYHEYY RRPVGVLLKK AQGWKRDRVA LNQEVMAPDA IKNFVPLLEA VSQAFVRMLH 190 200 210 220 230 240 GRVQQGVFSG DISDDLFRFA FESMTNIMFG ERLGMLEETV DPEAHEFIDA VYQMFHTSVP 250 260 270 280 290 300 MLSLPPSLFR LFRTRTWRDH VAAWDVIFTN ADKYTQSFYW DLRQKQDLGG SYRGILYSLL 310 320 330 340 350 360 GTSKLSFEDI KANVTEMLAG SVDTTSMTLQ WHLYEMGAAL GMQEMLRAEV LAARRQAQGD 370 380 390 400 410 420 MTAMLQSVPL LKASIKETLR LHPISVTLQR YLVNDLVLQD YMIPAKTLVQ VANYGMGREP 430 440 SFFANPEKFD PPRWLDKDKN ATHFR

References

[1]

"Inherited congenital adrenal hyperplasia in the rabbit is caused by a deletion in the gene encoding cytochrome P450 cholesterol side-chain cleavage enzyme." Yang X., Iwamoto K., Wang M., Artwohl J., Mason J.I., Pang S. Endocrinology 132:1977-1982(1993) [PubMed: 7682938] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA].

Cross-references

Sequence databases
EMBLGenBankDDBJ	S59219 mRNA. Translation: AAB26372.1.
PIR	A49189.
3D structure databases
HSSP	HSSP built from PDB template 1SCC based on UniProtKB P00189.
ModBase	Search...
Phylogenomic databases
HOVERGEN	Q28827.
Enzyme and pathway databases
BRENDA	1.14.15.6. 255.
Family and domain databases
InterPro	IPR001128. Cyt_P450. IPR017973. Cyt_P450_C. IPR017972. Cyt_P450_CS. IPR002399. Cyt_P450_mit_N. [Graphical view]
Gene3D	G3DSA:1.10.630.10. Cyt_P450. 1 hit.
PANTHER	PTHR19383. Cyt_P450. 1 hit.
Pfam	PF00067. p450. 1 hit. [Graphical view]
PRINTS	PR00408. MITP450. PR00385. P450.
PROSITE	PS00086. CYTOCHROME_P450. Partial match. [Graphical view]
ProtoNet	Search...

Entry information

Entry name

CP11A_RABIT

Accession

Primary (citable) accession number: Q28827

Entry history

Integrated into UniProtKB/Swiss-Prot:	December 15, 1998
Last sequence update:	November 1, 1996
Last modified:	October 13, 2009
This is version 54 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)