Cholesterol side-chain cleavage enzyme

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Cytochrome P450, family 11, subfamily A, polypeptide 1
Identifiers
Symbols CYP11A1; CYP11A; P450SCC
External IDs OMIM: 118485 MGI: 88582 HomoloGene: 37347
EC number 1.14.15.6
RNA expression pattern

More reference expression data
Orthologs
Human Mouse
Entrez 1583 13070
Ensembl ENSG00000140459 ENSMUSG00000032323
Uniprot P05108 Q6NV84
Refseq NM_000781 (mRNA)
NP_000772 (protein)		 XM_976231 (mRNA)
XP_981325 (protein)
Location Chr 15: 72.42 - 72.45 Mb Chr 9: 57.81 - 57.83 Mb
Pubmed search [1] [2]

Cytochrome P450, family 11, subfamily A, polypeptide 1, also known as CYP11A1, is a human gene.[1]

P450 scc (or 20,22-Desmolase) is an enzyme associated with the conversion of cholesterol to pregnenolone. The terminology "Desmolase" is becoming outdated and the enzyme is now more commonly referred to as P450scc.

The "scc" refers to side chain cleavage.

Contents

Regulation

The action of P450scc is primarily regulated by two pituitary hormones: LH and ACTH. Both increase the action of P450scc.

P450scc is also inhibited by the drug aminoglutethimide to block the steroid pathway.

Steroid hormone synthesis

Steroidogenesis, showing cholesterol side-chain cleavage enzyme at top.
Steroidogenesis, showing cholesterol side-chain cleavage enzyme at top.
Steroid hormone synthesis
Steroid hormone synthesis

Pathology

A deficiency can lead to lipoid congenital adrenal hyperplasia. However, this is very rare.

See also

References

  1. ^ "Entrez Gene: CYP11A1 cytochrome P450, family 11, subfamily A, polypeptide 1".

Further reading

  • Helmberg A (1993). "Twin genes and endocrine disease: CYP21 and CYP11B genes.". Acta Endocrinol. 129 (2): 97–108. PMID 8372604. 
  • Papadopoulos V, Amri H, Boujrad N, et al. (1997). "Peripheral benzodiazepine receptor in cholesterol transport and steroidogenesis.". Steroids 62 (1): 21–8. PMID 9029710. 
  • Stocco DM (2000). "Intramitochondrial cholesterol transfer.". Biochim. Biophys. Acta 1486 (1): 184–97. PMID 10856721. 
  • Kristensen VN, Kure EH, Erikstein B, et al. (2001). "Genetic susceptibility and environmental estrogen-like compounds.". Mutat. Res. 482 (1-2): 77–82. PMID 11535251. 
  • Strauss JF (2004). "Some new thoughts on the pathophysiology and genetics of polycystic ovary syndrome.". Ann. N. Y. Acad. Sci. 997: 42–8. PMID 14644808. 
  • Guo IC, Shih MC, Lan HC, et al. (2007). "Transcriptional regulation of human CYP11A1 in gonads and adrenals.". J. Biomed. Sci. 14 (4): 509–15. doi:10.1007/s11373-007-9177-z. PMID 17594537. 
  • Wada A, Waterman MR (1992). "Identification by site-directed mutagenesis of two lysine residues in cholesterol side chain cleavage cytochrome P450 that are essential for adrenodoxin binding.". J. Biol. Chem. 267 (32): 22877–82. PMID 1429635. 
  • Hu MC, Guo IC, Lin JH, Chung BC (1991). "Regulated expression of cytochrome P-450scc (cholesterol-side-chain cleavage enzyme) in cultured cell lines detected by antibody against bacterially expressed human protein.". Biochem. J. 274 ( Pt 3): 813–7. PMID 1849407. 
  • Sparkes RS, Klisak I, Miller WL (1991). "Regional mapping of genes encoding human steroidogenic enzymes: P450scc to 15q23-q24, adrenodoxin to 11q22; adrenodoxin reductase to 17q24-q25; and P450c17 to 10q24-q25.". DNA Cell Biol. 10 (5): 359–65. PMID 1863359. 
  • Coghlan VM, Vickery LE (1991). "Site-specific mutations in human ferredoxin that affect binding to ferredoxin reductase and cytochrome P450scc.". J. Biol. Chem. 266 (28): 18606–12. PMID 1917982. 
  • Matteson KJ, Chung BC, Urdea MS, Miller WL (1986). "Study of cholesterol side-chain cleavage (20,22 desmolase) deficiency causing congenital lipoid adrenal hyperplasia using bovine-sequence P450scc oligodeoxyribonucleotide probes.". Endocrinology 118 (4): 1296–305. PMID 2419119. 
  • Chung BC, Matteson KJ, Voutilainen R, et al. (1987). "Human cholesterol side-chain cleavage enzyme, P450scc: cDNA cloning, assignment of the gene to chromosome 15, and expression in the placenta.". Proc. Natl. Acad. Sci. U.S.A. 83 (23): 8962–6. PMID 3024157. 
  • Morohashi K, Sogawa K, Omura T, Fujii-Kuriyama Y (1987). "Gene structure of human cytochrome P-450(SCC), cholesterol desmolase.". J. Biochem. 101 (4): 879–87. PMID 3038854. 
  • Maruyama K, Sugano S (1994). "Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides.". Gene 138 (1-2): 171–4. PMID 8125298. 
  • Gharani N, Waterworth DM, Batty S, et al. (1997). "Association of the steroid synthesis gene CYP11a with polycystic ovary syndrome and hyperandrogenism.". Hum. Mol. Genet. 6 (3): 397–402. PMID 9147642. 
  • Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, et al. (1997). "Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library.". Gene 200 (1-2): 149–56. PMID 9373149. 
  • Hukkanen J, Mäntylä M, Kangas L, et al. (1998). "Expression of cytochrome P450 genes encoding enzymes active in the metabolism of tamoxifen in human uterine endometrium.". Pharmacol. Toxicol. 82 (2): 93–7. PMID 9498238. 
  • Zhou Z, Shackleton CH, Pahwa S, et al. (1998). "Prominent sex steroid metabolism in human lymphocytes.". Mol. Cell. Endocrinol. 138 (1-2): 61–9. PMID 9685215. 

Additional images

Cholesterol

Pregnenolone

External links

 This article on a gene on chromosome 15 is a stub. You can help Wikipedia by expanding it.
v • d • e
Cytochrome P450, oxygenases: steroid hydroxylases - steroidogenesis (EC 1.14)
To pregnenolone
Side-chain cleavage
To aldosterone/cortisol
To sex hormones
Other
v • d • e
Cytochromes, oxygenases: cytochrome P450 (EC 1.14)
CYP1
A1, A2, B1
CYP2
A6, A7, A13, B6, C8, C9, C18, C19, D6, E1, F1, J2, R1, S1, U1, W1
CYP3
CYP4
A11, A22, B1, F2, F3, F8, F11, F12, F22, V2, X1, Z1
CYP5-20
CYP5 (A1) - CYP7 (A1, B1) - CYP8 (A1, B1) - CYP11 (A1, B1, B2) - CYP17 (A1) - CYP19 (A1) - CYP20 (A1)
CYP21-51
CYP21 (A2) - CYP24 (A1) - CYP26 (A1, B1, C1) - CYP27 (A1, B1, C1) - CYP39 (A1) - CYP46 (A1) - CYP51 (A1)
v • d • e
Oxidoreductases: dioxygenases, including steroid hydroxylases (EC 1.14)
1.14.11 - 2-oxoglutarate
1.14.13 - NADH or NADPH
1.14.14 - reduced flavin or flavoprotein
1.14.15 - reduced iron-sulfur protein
11B1 - 11B2 - 11A1
1.14.16 - reduced pteridine
1.14.17 - reduced ascorbate
1.14.18-19 - other
1.14.99 - miscellaneous
v • d • e
Enzymes: multienzyme complexes
v • d • e
Mitochondrial enzymes and transporters
Outer membrane
Intermembrane space
Inner membrane
Matrix
Other/to be sorted
Cholesterol side-chain cleavage enzyme
Mitochondrial DNA

Wikipedia content modification information:

  • This page was last modified on 4 October 2008, at 13:32.

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