甲基丙二酸单酰辅酶A：修订间差异

甲基丙二酸单酰辅酶A
识别
CAS号	1264-45-5
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。

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2022年8月31日 (三) 03:04的版本

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]

生物合成及转换

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:

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

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。

另见

甲基丙二酸单酰辅酶A差向异构酶

参考文献

^ 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). ISSN 1096-7192. PMC 5725275 . PMID 29033250. doi:10.1016/j.ymgme.2017.10.002 （英语）. 引文格式1维护：PMC格式 (link)
^ ^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. 引文格式1维护：PMC格式 (link)
^ Nelson, David L.; Cox, Michael M. Lehninger principles of biochemistry. Fourth edition. 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)
^ 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）. 引文格式1维护：PMC格式 (link)
^ 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). ISSN 0141-8955. doi:10.1002/jimd.12009 （英语）.

[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). ISSN 1096-7192. PMC 5725275 . PMID 29033250. doi:10.1016/j.ymgme.2017.10.002 （英语）. 引文格式1维护：PMC格式 (link)

[:0-3] 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 . PMID 25205257. doi:10.1186/s13023-014-0130-8. 引文格式1维护：PMC格式 (link)

[4] Nelson, David L.; Cox, Michael M. Lehninger principles of biochemistry. Fourth edition. 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）.

[:1-6] 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 . PMID 22661206. doi:10.1631/jzus.B1100329 （英语）. 引文格式1维护：PMC格式 (link)

[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). ISSN 0141-8955. doi:10.1002/jimd.12009 （英语）.

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 ==生物合成及转换==
+由奇数碳的[[脂肪酸]][[代谢]]或[[膽固醇|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[[甲基丙二酸单酰辅酶A|甲基丙二酰辅酶A]]<ref>{{Cite journal |last=Wongkittichote |first=Parith |last2=Ah Mew |first2=Nicholas |last3=Chapman |first3=Kimberly A. |date=2017-12-01 |title=Propionyl-CoA carboxylase – A review |url=https://www.sciencedirect.com/science/article/pii/S1096719217305322 |journal=Molecular Genetics and Metabolism |language=en |volume=122 |issue=4 |doi=10.1016/j.ymgme.2017.10.002 |issn=1096-7192 |pmc=PMC5725275 |pmid=29033250}}</ref><ref name=":0">{{Cite journal |last=Baumgartner |first=Matthias R. |last2=Hörster |first2=Friederike |last3=Dionisi-Vici |first3=Carlo |last4=Haliloglu |first4=Goknur |last5=Karall |first5=Daniela |last6=Chapman |first6=Kimberly A. |last7=Huemer |first7=Martina |last8=Hochuli |first8=Michel |last9=Assoun |first9=Murielle |last10=Ballhausen |first10=Diana |last11=Burlina |first11=Alberto |date=2014-09-02 |title=Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia |url=https://doi.org/10.1186/s13023-014-0130-8 |journal=Orphanet Journal of Rare Diseases |volume=9 |issue=1 |doi=10.1186/s13023-014-0130-8 |issn=1750-1172 |pmc=PMC4180313 |pmid=25205257}}</ref>。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）[[异构化反应|异构化]]为[[琥珀酰辅酶A]]（以[[维生素B12|维生素B<sub>12</sub>]]为辅酶）。此[[异构化反应|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]]<ref>{{Cite book|edition=Fourth edition|chapter=Lehninger principles of biochemistry|url=https://www.worldcat.org/oclc/55476414|publisher=W.H. Freeman|date=2005|location=New York|isbn=0-7167-4339-6|oclc=55476414|first=David L.|last=Nelson|first2=Michael M.|last2=Cox}}</ref>。 即:
-[[丙酰辅酶A]]由[[丙酰辅酶A羧化酶]]羧化为甲基丙二酰辅酶A（以[[生物素]](维生素B<sub>7</sub>)为辅酶）。甲基丙二酰辅酶A再通过[[甲基丙二酸单酰辅酶A变位酶]]异构化为[[琥珀酰辅酶A]]（以[[维生素B12|维生素B<sub>12</sub>]]为辅酶），以这种方式进入[[三羧酸循环]]。 即:
-:[[丙酰辅酶A]] → 甲基丙二酰辅酶A → [[琥珀酰辅酶A]]
+:[[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B<sub>12</sub> ==
+[[维生素B12|维生素B<sub>12</sub>]]在此反应中起重要作用。[[辅酶B12|辅酶B<sub>12</sub>]]（[[辅酶B12|腺苷钴胺素]]）是维生素B<sub>12</sub>的一种[[有机金属化学|有机金属]]形式。它是[[甲基丙二酸单酰辅酶A变位酶|甲基丙二酰辅酶A变位酶]]的[[辅因子]]<ref>{{Cite book|chapter=Biochemistry of B12-Cofactors in Human Metabolism|title=Water Soluble Vitamins: Clinical Research and Future Application|url=https://doi.org/10.1007/978-94-007-2199-9_17|publisher=Springer Netherlands|date=2012|location=Dordrecht|isbn=978-94-007-2199-9|pages=323–346|doi=10.1007/978-94-007-2199-9_17|language=en|first=Bernhard|last=Kräutler|editor-first=Olaf|editor-last=Stanger}}</ref>。甲基丙二酰辅酶A转化为[[琥珀酰辅酶A]]是[[自由基|自由基反应]]。
+== 相关疾病 ==
+=== 甲基丙二酸血症（Methylmalonic Acidemia，MMA） ===
+此疾病发生于[[甲基丙二酸单酰辅酶A变位酶|甲基丙二酰辅酶A变位酶]]（MUT）无法将足够的[[甲基丙二酸单酰辅酶A|甲基丙二酰辅酶A]]转化为[[琥珀酰辅酶A]]<ref name=":1">{{Cite journal |last=Takahashi-Iñiguez |first=Tóshiko |last2=García-Hernandez |first2=Enrique |last3=Arreguín-Espinosa |first3=Roberto |last4=Flores |first4=María Elena |date=2012-06-01 |title=Role of vitamin B12 on methylmalonyl-CoA mutase activity |url=https://doi.org/10.1631/jzus.B1100329 |journal=Journal of Zhejiang University SCIENCE B |language=en |volume=13 |issue=6 |doi=10.1631/jzus.B1100329 |issn=1862-1783 |pmc=PMC3370288 |pmid=22661206}}</ref>，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<ref name=":0" />。此疾病与[[维生素B12|维生素B<sub>12</sub>]]有关，因为[[维生素B12|维生素B<sub>12</sub>]]是[[甲基丙二酸单酰辅酶A变位酶|甲基丙二酰辅酶A变位酶]]（MUT）的[[辅因子]]<ref>{{Cite journal |last=Froese |first=D. Sean |last2=Fowler |first2=Brian |last3=Baumgartner |first3=Matthias R. |date=2019-07 |title=Vitamin B 12 , folate, and the methionine remethylation cycle—biochemistry, pathways, and regulation |url=https://onlinelibrary.wiley.com/doi/10.1002/jimd.12009 |journal=Journal of Inherited Metabolic Disease |language=en |volume=42 |issue=4 |doi=10.1002/jimd.12009 |issn=0141-8955}}</ref><ref name=":1" />。
 ==另见==