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Identification
BMDB Protein ID BMDBP00053
Secondary Accession Numbers
  • BMDBP03121
Name Cholesterol side-chain cleavage enzyme, mitochondrial
Synonyms Not Available
Gene Name CYP11A1
Protein Type Enzyme
Biological Properties
General Function Secondary metabolites biosynthesis, transport and catabolism
Specific Function A cytochrome P450 monooxygenase that catalyzes the side-chain hydroxylation and cleavage of cholesterol to pregnenolone, the precursor of most steroid hormones (PubMed:11412116). Catalyzes three sequential oxidation reactions of cholesterol, namely the hydroxylation at C22 followed with the hydroxylation at C20 to yield 20R,22R-hydroxycholesterol that is further cleaved between C20 and C22 to yield the C21-steroid pregnenolone and 4-methylpentanal (PubMed:11412116). Mechanistically, uses molecular oxygen inserting one oxygen atom into a substrate and reducing the second into a water molecule. Two electrons are provided by NADPH via a two-protein mitochondrial transfer system comprising flavoprotein FDXR (adrenodoxin/ferredoxin reductase) and nonheme iron-sulfur protein FDX1 or FDX2 (adrenodoxin/ferredoxin) (PubMed:11412116).
Pathways
  • C21-steroid hormone metabolism
  • Lipid metabolism
Reactions
3a,7a-Dihydroxy-5b-cholestane + 3 NADPH + 3 Oxygen → 3 alpha,7 alpha,26-Trihydroxy-5beta-cholestane +3 NADP +3 Water details
3 alpha,7 alpha,26-Trihydroxy-5beta-cholestane → 3a,7a-Dihydroxy-5b-cholestan-26-al details
3a,7a-Dihydroxy-5b-cholestan-26-al → 3a,7a-Dihydroxycoprostanic acid details
5-b-Cholestane-3a ,7a ,12a-triol + 3 NADPH + 3 Oxygen → 27-Deoxy-5b-cyprinol +3 NADP +4 Water details
27-Deoxy-5b-cyprinol + 3 NADPH + 3 Oxygen → 3a,7a,12a-Trihydroxy-5b-cholestan-26-al +3 NADP +4 Water details
3a,7a,12a-Trihydroxy-5b-cholestan-26-al → Coprocholic acid details
27-Hydroxycholesterol + 3 NADPH + 3 Oxygen → 3beta-Hydroxy-5-cholestenoic acid +3 NADP +4 Water details
GO Classification Not Available
Cellular Location Not Available
Gene Properties
Chromosome Location 21
Locus Not Available
SNPs CYP11A1
Gene Sequence
>1563 bp
.AGCAGCAGCTGTGGGGGCAGCATGCTAGCAAGGGGGCTTCCCCTCCGCTCAGCCCTGGT
CAAAGCCTGCCCACCCATCCTGAGCACAGTGGGGGAGGGCTGGGGCCACCACAGGGTGGG
CACTGGAGAGGGAGCTGGCATCTCCACAAAGACCCCTCGCCCCTACAGTGAGATCCCCTC
CCCTGGTGACAATGGCTGGCTTAACCTCTACCATTTCTGGAGGGAGAAGGGCTCACAGAG
AATCCACTTTCGCCACATCGAGAACTTCCAGAAGTATGGCCCCATTTACAGGGAGAAGCT
TGGCAATTTGGAGTCAGTTTATATCATTCACCCTGAAGACGTGGCCCATCTCTTCAAGTT
CGAGGGATCCTACCCAGAGAGATATGACATCCCGCCCTGGCTGGCCTATCACCGATATTA
TCAGAAACCCATTGGAGTCCTGTTTAAGAAGTCAGGAACCTGGAAGAAAGACCGGGTGGT
CCTGAACACGGAGGTGATGGCTCCAGAGGCAATAAAGAACTTCATCCCACTGCTGAATCC
AGTGTCTCAGGACTTCGTCAGCCTCCTGCACAAGCGCATCAAGCAGCAGGGCTCCGGAAA
GTTTGTAGGGGACATCAAGGAAGACCTGTTTCACTTTGCCTTTGAGTCCATCACCAATGT
CATGTTTGGGGAGCGCCTGGGGATGCTGGAGGAGACAGTGAACCCCGAGGCCCAGAAGTT
CATTGATGCCGTCTACAAGATGTTCCACACCAGTGTCCCTCTGCTCAACGTCCCTCCAGA
ACTGTACCGTCTATTCAGAACCAAGACTTGGAGGGACCATGTAGCCGCATGGGACACAAT
TTTCAATAAAGCTGAAAAATACACTGAGATCTTCTACCAGGACCTGAGACGGAAAACAGA
ATTTAGGAATTACCCAGGCATCCTCTACTGCCTCCTGAAAAGTGAGAAGATGCTCTTGGA
GGATGTCAAGGCCAATATTACGGAGATGCTGGCAGGGGGTGTGAACACGACATCCATGAC
ATTGCAATGGCACTTGTACGAGATGGCACGCAGCCTGAATGTGCAGGAGATGCTGCGGGA
GGAGGTTCTGAATGCCCGACGCCAGGCAGAGGGAGACATAAGCAAGATGCTGCAAATGGT
CCCACTTCTCAAAGCTAGCATCAAGGAGACGCTGAGACTCCACCCCATCTCCGTGACCCT
GCAGAGATACCCTGAAAGTGACTTGGTTCTTCAAGATTACCTGATTCCTGCCAAGACACT
GGTGCAAGTGGCCATCTATGCCATGGGCCGAGACCCTGCCTTCTTCTCCAGTCCGGACAA
GTTTGACCCAACCAGGTGGCTGAGTAAAGACAAAGACCTCATCCACTTCCGGAACCTGGG
CTTTGGCTGGGGAGTGCGGCAGTGCGTGGGCCGGCGGATCGCCGAGCTGGAGATGACCCT
CTTCCTCATCCACATTCTGGAGAACTTCAAGGTTGAAATGCAGCATATCGGTGACGTGGA
CACCATATTCAACCTCATCCTGACGCCGGACAAGCCCATCTTCCTTGTCTTCCGCCCCTT
CAA
Protein Properties
Number of Residues 520
Molecular Weight 60333.0
Theoretical pI 9.15
Pfam Domain Function Not Available
Signals
  • None
Transmembrane Regions Not Available
Protein Sequence
>Cholesterol side-chain cleavage enzyme, mitochondrial
MLARGLPLRSALVKACPPILSTVGEGWGHHRVGTGEGAGISTKTPRPYSEIPSPGDNGWL
NLYHFWREKGSQRIHFRHIENFQKYGPIYREKLGNLESVYIIHPEDVAHLFKFEGSYPER
YDIPPWLAYHRYYQKPIGVLFKKSGTWKKDRVVLNTEVMAPEAIKNFIPLLNPVSQDFVS
LLHKRIKQQGSGKFVGDIKEDLFHFAFESITNVMFGERLGMLEETVNPEAQKFIDAVYKM
FHTSVPLLNVPPELYRLFRTKTWRDHVAAWDTIFNKAEKYTEIFYQDLRRKTEFRNYPGI
LYCLLKSEKMLLEDVKANITEMLAGGVNTTSMTLQWHLYEMARSLNVQEMLREEVLNARR
QAEGDISKMLQMVPLLKASIKETLRLHPISVTLQRYPESDLVLQDYLIPAKTLVQVAIYA
MGRDPAFFSSPDKFDPTRWLSKDKDLIHFRNLGFGWGVRQCVGRRIAELEMTLFLIHILE
NFKVEMQHIGDVDTIFNLILTPDKPIFLVFRPFNQDPPQA
GenBank ID Protein AAA30488.1
UniProtKB/Swiss-Prot ID P00189
UniProtKB/Swiss-Prot Entry Name CP11A_BOVIN
PDB IDs
GenBank Gene ID K02130
GeneCard ID CYP11A1
GenAtlas ID Not Available
HGNC ID Not Available
References
General References Not Available