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{{PBB|geneid=4594}} {{enzyme | Name = 甲基丙二酸單酰輔酶A變位酶 | EC_number = 5.4.99.2 | CAS_number = 9023-90-9 | IUBMB_EC_number = 5/4/99/2 | GO_code = 0004494 }} '''甲基丙二酸單酰輔酶A變位酶'''（{{lang-en|Methylmalonyl Coenzyme-A mutase}}，MCM，亦簡稱'''[[甲基丙二酰輔酶A]][[變位酶]]'''）是一種在[[代謝途徑]]上將[[甲基丙二酸]]單酰[[輔酶A]]轉(zhuǎn)換為[[琥珀酰輔酶A]]的酶，同時需要[[維生素B12|維生素B₁₂]]的幫助。 ==功能== 甲基丙二酰輔酶A變位酶主要分布在[[線粒體]]中，[[催化]]甲基丙二酰輔酶A異構(gòu)為琥珀酰輔酶A從而進入[[三羧酸循環(huán)]]。而甲基丙二酰輔酶A來源于[[丙酰輔酶A]]，后者是[[異亮氨酸]]、[[纈氨酸]]、[[蘇氨酸]]、[[甲硫氨酸]]、[[胸腺嘧啶]]、[[膽固醇]]以及奇數(shù)鏈[[脂肪酸]][[分解代謝]]的共同中間體。 ==人類[[基因組]]== 人類基因組中編碼該酶的[[基因]]縮寫為'''MUT'''。<ref name="entrez">{{cite web | title = Entrez Gene: MUT methylmalonyl Coenzyme A mutase| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=4594| accessdate = }}</ref> ==參考文獻== {{reflist}} ==進一步閱讀== {{refbegin | 2}} {{PBB_Further_reading | citations = *{{cite journal | author=Ledley FD, Rosenblatt DS |title=Mutations in mut methylmalonic acidemia: clinical and enzymatic correlations. |journal=Hum. Mutat. |volume=9 |issue= 1 |pages= 1–6 |year= 1997 |pmid= 8990001 |doi= 10.1002/(SICI)1098-1004(1997)9:1<1::AID-HUMU1>3.0.CO;2-E }} *{{cite journal | author=Ludwig ML, Matthews RG |title=Structure-based perspectives on B12-dependent enzymes. |journal=Annu. Rev. Biochem. |volume=66 |issue= |pages= 269–313 |year= 1997 |pmid= 9242908 |doi= 10.1146/annurev.biochem.66.1.269 }} *{{cite journal | author=Lubrano R, Elli M, Rossi M, ''et al.'' |title=Renal transplant in methylmalonic acidemia: could it be the best option? Report on a case at 10 years and review of the literature. |journal=Pediatr. Nephrol. |volume=22 |issue= 8 |pages= 1209–14 |year= 2007 |pmid= 17401587 |doi= 10.1007/s00467-007-0460-z }} *{{cite journal | author=Frenkel EP, Kitchens RL |title=Intracellular localization of hepatic propionyl-CoA carboxylase and methylmalonyl-CoA mutase in humans and normal and vitamin B12 deficient rats. |journal=Br. J. Haematol. |volume=31 |issue= 4 |pages= 501–13 |year= 1978 |pmid= 24458 |doi=10.1111/j.1365-2141.1975.tb00885.x }} *{{cite journal | author=Crane AM, Jansen R, Andrews ER, Ledley FD |title=Cloning and expression of a mutant methylmalonyl coenzyme A mutase with altered cobalamin affinity that causes mut- methylmalonic aciduria. |journal=J. Clin. Invest. |volume=89 |issue= 2 |pages= 385–91 |year= 1992 |pmid= 1346616 |doi=10.1172/JCI115597 | pmc=442864 }} *{{cite journal | author=Crane AM, Martin LS, Valle D, Ledley FD |title=Phenotype of disease in three patients with identical mutations in methylmalonyl CoA mutase. |journal=Hum. Genet. |volume=89 |issue= 3 |pages= 259–64 |year= 1992 |pmid= 1351030 |doi=10.1007/BF00220536 }} *{{cite journal | author=Raff ML, Crane AM, Jansen R, ''et al.'' |title=Genetic characterization of a MUT locus mutation discriminating heterogeneity in mut0 and mut- methylmalonic aciduria by interallelic complementation. |journal=J. Clin. Invest. |volume=87 |issue= 1 |pages= 203–7 |year= 1991 |pmid= 1670635 |doi=10.1172/JCI114972 | pmc=295026 }} *{{cite journal | author=Jansen R, Ledley FD |title=Heterozygous mutations at the mut locus in fibroblasts with mut0 methylmalonic acidemia identified by polymerase-chain-reaction cDNA cloning. |journal=Am. J. Hum. Genet. |volume=47 |issue= 5 |pages= 808–14 |year= 1990 |pmid= 1977311 |doi= | pmc=1683687 }} *{{cite journal | author=Nham SU, Wilkemeyer MF, Ledley FD |title=Structure of the human methylmalonyl-CoA mutase (MUT) locus. |journal=Genomics |volume=8 |issue= 4 |pages= 710–6 |year= 1991 |pmid= 1980486 |doi=10.1016/0888-7543(90)90259-W }} *{{cite journal | author=Ledley FD, Lumetta M, Nguyen PN, ''et al.'' |title=Molecular cloning of L-methylmalonyl-CoA mutase: gene transfer and analysis of mut cell lines. |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=85 |issue= 10 |pages= 3518–21 |year= 1988 |pmid= 2453061 |doi=10.1073/pnas.85.10.3518 | pmc=280243 }} *{{cite journal | author=Jansen R, Kalousek F, Fenton WA, ''et al.'' |title=Cloning of full-length methylmalonyl-CoA mutase from a cDNA library using the polymerase chain reaction. |journal=Genomics |volume=4 |issue= 2 |pages= 198–205 |year= 1989 |pmid= 2567699 |doi=10.1016/0888-7543(89)90300-5 }} *{{cite journal | author=Fenton WA, Hack AM, Kraus JP, Rosenberg LE |title=Immunochemical studies of fibroblasts from patients with methylmalonyl-CoA mutase apoenzyme deficiency: detection of a mutation interfering with mitochondrial import. |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=84 |issue= 5 |pages= 1421–4 |year= 1987 |pmid= 2881300 |doi=10.1073/pnas.84.5.1421 | pmc=304442 }} *{{cite journal | author=Zoghbi HY, O'Brien WE, Ledley FD |title=Linkage relationships of the human methylmalonyl CoA mutase to the HLA and D6S4 loci on chromosome 6. |journal=Genomics |volume=3 |issue= 4 |pages= 396–8 |year= 1989 |pmid= 2907507 |doi=10.1016/0888-7543(88)90135-8 }} *{{cite journal | author=Kolhouse JF, Utley C, Allen RH |title=Isolation and characterization of methylmalonyl-CoA mutase from human placenta. |journal=J. Biol. Chem. |volume=255 |issue= 7 |pages= 2708–12 |year= 1980 |pmid= 6102092 |doi= }} *{{cite journal | author=Fenton WA, Hack AM, Willard HF, ''et al.'' |title=Purification and properties of methylmalonyl coenzyme A mutase from human liver. |journal=Arch. Biochem. Biophys. |volume=214 |issue= 2 |pages= 815–23 |year= 1982 |pmid= 6124211 |doi=10.1016/0003-9861(82)90088-1 }} *{{cite journal | author=Qureshi AA, Crane AM, Matiaszuk NV, ''et al.'' |title=Cloning and expression of mutations demonstrating intragenic complementation in mut0 methylmalonic aciduria. |journal=J. Clin. Invest. |volume=93 |issue= 4 |pages= 1812–9 |year= 1994 |pmid= 7909321 |doi=10.1172/JCI117166 | pmc=294249 }} *{{cite journal | author=Crane AM, Ledley FD |title=Clustering of mutations in methylmalonyl CoA mutase associated with mut- methylmalonic acidemia. |journal=Am. J. Hum. Genet. |volume=55 |issue= 1 |pages= 42–50 |year= 1994 |pmid= 7912889 |doi= | pmc=1918235 }} *{{cite journal | author=Janata J, Kogekar N, Fenton WA |title=Expression and kinetic characterization of methylmalonyl-CoA mutase from patients with the mut- phenotype: evidence for naturally occurring interallelic complementation. |journal=Hum. Mol. Genet. |volume=6 |issue= 9 |pages= 1457–64 |year= 1998 |pmid= 9285782 |doi=10.1093/hmg/6.9.1457 }} }} {{refend}} ==外部鏈接== * [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=gene&part=mma GeneReviews/NIH/NCBI/UW entry on Methylmalonic Acidemia] * {{MeshName|Methylmalonyl-CoA+Mutase}} {{變位酶}} {{Amino acid metabolism enzymes}} {{三羧酸循環(huán)酶類與電子傳遞鏈}} <!-- The PBB_Controls template provides controls for Protein Box Bot, please see Template:PBB_Controls for details. --> {{PBB_Controls | update_page = yes | require_manual_inspection = no | update_protein_box = yes | update_summary = no | update_citations = yes }} [[Category:酶]] ==參考來源== *[http://zh.wikipedia.org/wiki/%E7%94%B2%E5%9F%BA%E4%B8%99%E4%BA%8C%E9%85%B8%E5%8D%95%E9%85%B0%E8%BE%85%E9%85%B6A%E5%8F%98%E4%BD%8D%E9%85%B6 維基百科-甲基丙二酸單酰輔酶A變位酶]

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