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甲基丙二酸单酰辅酶A

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甲基丙二酸单酰辅酶A
识别
CAS号 1264-45-5  checkY
PubChem 123909
ChemSpider 110440
InChI
 
  • 1/C25H40N7O19P3S/c1-12(23(37)38)24(39)55-7-6-27-14(33)4-5-28-21(36)18(35)25(2,3)9-48-54(45,46)51-53(43,44)47-8-13-17(50-52(40,41)42)16(34)22(49-13)32-11-31-15-19(26)29-10-30-20(15)32/h10-13,16-18,22,34-35H,4-9H2,1-3H3,(H,27,33)(H,28,36)(H,37,38)(H,43,44)(H,45,46)(H2,26,29,30)(H2,40,41,42)/t12?,13-,16-,17-,18+,22-/m1/s1
InChIKey MZFOKIKEPGUZEN-FBMOWMAEBZ
ChEBI 16625
MeSH methylmalonyl-coenzyme+A
IUPHAR配体 5223
性质
化学式 C25H40N7O19P3S
摩尔质量 867.608 g/mol g·mol⁻¹
若非注明,所有数据均出自标准状态(25 ℃,100 kPa)下。

甲基丙二酸单酰辅酶A (或简称甲基丙二酰辅酶A)是由辅酶A甲基丙二酸通过硫酯键结合的重要代谢中间产物,参与许多生物合成与分解反应。[1]

生物合成及转换

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由奇数碳的脂肪酸代谢胆固醇侧链形成的丙酰辅酶A碳酸氢盐丙酰辅酶A羧化酶催化形成甲基丙二酰辅酶A[2][3]。甲基丙二酰辅酶A再通过甲基丙二酸单酰辅酶A变位酶(methylmalonyl-CoA mutase,MUT)异构化琥珀酰辅酶A(以维生素B12为辅酶)。此异构化的反应是可逆的,其产物琥珀酰辅酶A进入三羧酸循环[4]。 即:

丙酰辅酶A + 碳酸氢盐 → 甲基丙二酰辅酶A → 琥珀酰辅酶A

维生素B12

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维生素B12在此反应中起重要作用。辅酶B12腺苷钴胺素)是维生素B12的一种有机金属形式。它是甲基丙二酰辅酶A变位酶辅因子[5]。甲基丙二酰辅酶A转化为琥珀酰辅酶A自由基反应

相关疾病

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甲基丙二酸血症(Methylmalonic Acidemia,MMA)

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此疾病发生于甲基丙二酰辅酶A变位酶(MUT)无法将足够的甲基丙二酰辅酶A转化为琥珀酰辅酶A[6],因此丙酸和/或甲基丙二酸在体内的发生异常积累,将可能导致婴儿严重的脑损伤。甚至死亡[3]。此疾病与维生素B12有关,因为维生素B12甲基丙二酰辅酶A变位酶(MUT)的辅因子[7][6]

另见

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参考文献

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  1. ^ Tabita, F. R., "The hydroxypropionate pathway of CO2 fixation: Fait accompli", Proceedings of the National Academy of Sciences 2009, vol. 106, 21015-21016. doi:10.1073/pnas.0912486107
  2. ^ Wongkittichote, Parith; Ah Mew, Nicholas; Chapman, Kimberly A. Propionyl-CoA carboxylase – A review. Molecular Genetics and Metabolism. 2017-12-01, 122 (4) [2022-08-31]. ISSN 1096-7192. PMC 5725275可免费查阅. PMID 29033250. doi:10.1016/j.ymgme.2017.10.002. (原始内容存档于2022-10-11) (英语). 
  3. ^ 3.0 3.1 Baumgartner, Matthias R.; Hörster, Friederike; Dionisi-Vici, Carlo; Haliloglu, Goknur; Karall, Daniela; Chapman, Kimberly A.; Huemer, Martina; Hochuli, Michel; Assoun, Murielle; Ballhausen, Diana; Burlina, Alberto. Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia. Orphanet Journal of Rare Diseases. 2014-09-02, 9 (1). ISSN 1750-1172. PMC 4180313可免费查阅. PMID 25205257. doi:10.1186/s13023-014-0130-8. 
  4. ^ Nelson, David L.; Cox, Michael M. Lehninger principles of biochemistry. Fourth. New York: W.H. Freeman https://www.worldcat.org/oclc/55476414. 2005. ISBN 0-7167-4339-6. OCLC 55476414.  缺少或|title=为空 (帮助)
  5. ^ Kräutler, Bernhard. Biochemistry of B12-Cofactors in Human Metabolism. Stanger, Olaf (编). Water Soluble Vitamins: Clinical Research and Future Application. Dordrecht: Springer Netherlands. 2012: 323–346. ISBN 978-94-007-2199-9. doi:10.1007/978-94-007-2199-9_17 (英语). 
  6. ^ 6.0 6.1 Takahashi-Iñiguez, Tóshiko; García-Hernandez, Enrique; Arreguín-Espinosa, Roberto; Flores, María Elena. Role of vitamin B12 on methylmalonyl-CoA mutase activity. Journal of Zhejiang University SCIENCE B. 2012-06-01, 13 (6). ISSN 1862-1783. PMC 3370288可免费查阅. PMID 22661206. doi:10.1631/jzus.B1100329 (英语). 
  7. ^ Froese, D. Sean; Fowler, Brian; Baumgartner, Matthias R. Vitamin B 12 , folate, and the methionine remethylation cycle—biochemistry, pathways, and regulation. Journal of Inherited Metabolic Disease. 2019-07, 42 (4) [2022-08-31]. ISSN 0141-8955. doi:10.1002/jimd.12009. (原始内容存档于2022-03-19) (英语).