甲基丙二酸单酰辅酶A
化合物
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与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]
甲基丙二酸单酰辅酶A | |
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识别 | |
CAS号 | 1264-45-5 ![]() |
PubChem | 123909 |
ChemSpider | 110440 |
InChI |
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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。 |
生物合成及转换
编辑由奇数碳的脂肪酸代谢或胆固醇侧链形成的丙酰辅酶A与碳酸氢盐经丙酰辅酶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)异构化为琥珀酰辅酶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:
维生素B12
编辑维生素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变位酶的辅因子[5]。甲基丙二酰辅酶A转化为琥珀酰辅酶A是自由基反应。
相关疾病
编辑甲基丙二酸血症(Methylmalonic Acidemia,MMA)
编辑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是甲基丙二酰辅酶A变位酶(MUT)的辅因子[7][6]。
另见
编辑参考文献
编辑- ^ 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
- ^ 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 . PMID 29033250. doi:10.1016/j.ymgme.2017.10.002. (原始内容存档于2022-10-11) (英语).
- ^ 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 . PMID 25205257. doi:10.1186/s13023-014-0130-8.
- ^ 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
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为空 (帮助) - ^ 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).
- ^ 6.0 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 . PMID 22661206. doi:10.1631/jzus.B1100329 (英语).
- ^ 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).