胆固醇侧链裂解酶

胆固醇侧链裂解酶
已知的結構 PDB 直系同源搜索: PDBe RCSB PDBID列表 3NA1、​3N9Y、​3N9Z、​3NA0
識別號
别名	CYP11A1；, CYP11A, CYPXIA1, P450SCC, cytochrome P450 family 11 subfamily A member 1
外部ID	OMIM：118485 MGI：88582 HomoloGene：37347 GeneCards：CYP11A1
相關疾病
congenital adrenal insufficiency[1]
為以下藥物的標靶
米托坦、​氨鲁米特[2]
基因位置（人类） 染色体 15號染色體[3] 基因座 15q24.1 起始 74,337,759 bp[3] 终止 74,367,646 bp[3]
基因位置（小鼠） 染色体 小鼠9号染色体[4] 基因座 9 B|9 31.63 cM 起始 57,913,694 bp[4] 终止 57,934,306 bp[4]
RNA表达模式
查阅更多表达数据
基因本體 分子功能 • iron ion binding • 金屬離子結合 • monooxygenase activity • heme binding • oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen • oxidoreductase activity • cholesterol monooxygenase (side-chain-cleaving) activity • 血浆蛋白结合 細胞組分 • 膜 • 线粒体基质 • 線粒體 • mitochondrial inner membrane 生物學過程 • vitamin D metabolic process • C21-steroid hormone metabolic process • 脂質代謝 • cholesterol metabolic process • C21-steroid hormone biosynthetic process • sterol metabolic process • steroid biosynthetic process • steroid metabolic process • glucocorticoid biosynthetic process • cellular response to peptide hormone stimulus • cortisol metabolic process Sources:Amigo / QuickGO
直系同源
物種	人類	小鼠
Entrez	1583	13070
Ensembl	ENSG00000140459 ​ENSG00000288362	ENSMUSG00000032323
UniProt	P05108	Q9QZ82
mRNA​序列	NM_001099773 ​NM_000781	NM_019779 ​NM_001346787
蛋白序列	NP_000772 ​NP_001093243	NP_001333716 ​NP_062753
基因位置​（UCSC）	Chr 15: 74.34 – 74.37 Mb	Chr 9: 57.91 – 57.93 Mb
PubMed​查找	[5]	[6]
維基數據
檢視/編輯人類 檢視/編輯小鼠

胆固醇侧链裂解酶（英語：Cholesterol side-chain cleavage enzyme，CYP11A1或P450scc，其中“scc”是“side-chain 侧链”“cleavage 裂解”的缩写）是一种将胆固醇转化为孕烯醇酮的线粒体酶。这是所有哺乳动物中由胆固醇生成各种甾类激素的第一步反应[7]。

胆固醇 + 3 NADPH + 3 H^+ + 3 O₂ ⇄ 孕烯醇酮 + 4-甲基戊醛 + 3 NADP^+ + 3 H₂O

P450scc 是细胞色素P450氧化酶超家族的一员（家族11亚家族A多肽1），基因名为 CYP11A1[8]。

另见

• 类固醇生成酶
• 细胞色素P450

参考文献

1. 與胆固醇侧链裂解酶相關的疾病；在維基數據上查看/編輯參考. 
2. 對胆固醇侧链裂解酶起作用的藥物；在維基數據上查看/編輯參考. 
3. GRCh38: Ensembl release 89: ENSG00000140459、​ENSG00000288362 - Ensembl, May 2017
4. GRCm38: Ensembl release 89: ENSMUSG00000032323 - Ensembl, May 2017
5. Human PubMed Reference:. National Center for Biotechnology Information, U.S. National Library of Medicine. 
6. Mouse PubMed Reference:. National Center for Biotechnology Information, U.S. National Library of Medicine. 
7. Hanukoglu I. Steroidogenic enzymes: structure, function, and role in regulation of steroid hormone biosynthesis. The Journal of Steroid Biochemistry and Molecular Biology. December 1992, 43 (8): 779–804. PMID 22217824. doi:10.1016/0960-0760(92)90307-5. 
8. Entrez Gene: CYP11A1 cytochrome P450, family 11, subfamily A, polypeptide 1. 

延伸阅读

• Helmberg A. Twin genes and endocrine disease: CYP21 and CYP11B genes. Acta Endocrinologica. August 1993, 129 (2): 97–108. PMID 8372604. doi:10.1530/acta.0.1290097. 
• Papadopoulos V, Amri H, Boujrad N, Cascio C, Culty M, Garnier M, Hardwick M, Li H, Vidic B, Brown AS, Reversa JL, Bernassau JM, Drieu K. Peripheral benzodiazepine receptor in cholesterol transport and steroidogenesis. Steroids. January 1997, 62 (1): 21–8. PMID 9029710. doi:10.1016/S0039-128X(96)00154-7. 
• Stocco DM. Intramitochondrial cholesterol transfer. Biochimica et Biophysica Acta. June 2000, 1486 (1): 184–97. PMID 10856721. doi:10.1016/S1388-1981(00)00056-1. 
• Kristensen VN, Kure EH, Erikstein B, Harada N, Børresen-Dale A. Genetic susceptibility and environmental estrogen-like compounds. Mutation Research. October 2001, 482 (1–2): 77–82. PMID 11535251. doi:10.1016/S0027-5107(01)00212-3. 
• Strauss JF. Some new thoughts on the pathophysiology and genetics of polycystic ovary syndrome. Annals of the New York Academy of Sciences. November 2003, 997 (1): 42–8. PMID 14644808. doi:10.1196/annals.1290.005. 
• Wada A, Waterman MR. Identification by site-directed mutagenesis of two lysine residues in cholesterol side chain cleavage cytochrome P450 that are essential for adrenodoxin binding. The Journal of Biological Chemistry. November 1992, 267 (32): 22877–82. PMID 1429635. 
• Hu MC, Guo IC, Lin JH, Chung BC. Regulated expression of cytochrome P-450scc (cholesterol-side-chain cleavage enzyme) in cultured cell lines detected by antibody against bacterially expressed human protein. The Biochemical Journal. March 1991, 274 (Pt 3): 813–7. PMC 1149983 . PMID 1849407. doi:10.1042/bj2740813. 
• Sparkes RS, Klisak I, Miller WL. 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 and Cell Biology. June 1991, 10 (5): 359–65. PMID 1863359. doi:10.1089/dna.1991.10.359. 
• Coghlan VM, Vickery LE. Site-specific mutations in human ferredoxin that affect binding to ferredoxin reductase and cytochrome P450scc. The Journal of Biological Chemistry. October 1991, 266 (28): 18606–12. PMID 1917982. 
• Matteson KJ, Chung BC, Urdea MS, Miller WL. Study of cholesterol side-chain cleavage (20,22 desmolase) deficiency causing congenital lipoid adrenal hyperplasia using bovine-sequence P450scc oligodeoxyribonucleotide probes. Endocrinology. April 1986, 118 (4): 1296–305. PMID 2419119. doi:10.1210/endo-118-4-1296. 
• Chung BC, Matteson KJ, Voutilainen R, Mohandas TK, Miller WL. Human cholesterol side-chain cleavage enzyme, P450scc: cDNA cloning, assignment of the gene to chromosome 15, and expression in the placenta. Proceedings of the National Academy of Sciences of the United States of America. December 1986, 83 (23): 8962–6. PMC 387054 . PMID 3024157. doi:10.1073/pnas.83.23.8962. 
• Morohashi K, Sogawa K, Omura T, Fujii-Kuriyama Y. Gene structure of human cytochrome P-450(SCC), cholesterol desmolase. Journal of Biochemistry. April 1987, 101 (4): 879–87. PMID 3038854. 
• Maruyama K, Sugano S. Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides. Gene. January 1994, 138 (1–2): 171–4. PMID 8125298. doi:10.1016/0378-1119(94)90802-8. 
• Gharani N, Waterworth DM, Batty S, White D, Gilling-Smith C, Conway GS, McCarthy M, Franks S, Williamson R. Association of the steroid synthesis gene CYP11a with polycystic ovary syndrome and hyperandrogenism. Human Molecular Genetics. March 1997, 6 (3): 397–402. PMID 9147642. doi:10.1093/hmg/6.3.397. 
• Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, Suyama A, Sugano S. Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library. Gene. October 1997, 200 (1–2): 149–56. PMID 9373149. doi:10.1016/S0378-1119(97)00411-3. 
• Hukkanen J, Mäntylä M, Kangas L, Wirta P, Hakkola J, Paakki P, Evisalmi S, Pelkonen O, Raunio H. Expression of cytochrome P450 genes encoding enzymes active in the metabolism of tamoxifen in human uterine endometrium. Pharmacology & Toxicology. February 1998, 82 (2): 93–7. PMID 9498238. doi:10.1111/j.1600-0773.1998.tb01404.x. 
• Zhou Z, Shackleton CH, Pahwa S, White PC, Speiser PW. Prominent sex steroid metabolism in human lymphocytes. Molecular and Cellular Endocrinology. March 1998, 138 (1–2): 61–9. PMID 9685215. doi:10.1016/S0303-7207(98)00052-5.