甲基丙二酸單醯輔酶A

甲基丙二酸單醯輔酶A
識別
CAS號	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配體	5223
性質
化學式	C25H40N7O19P3S
莫耳質量	867.608 g/mol g·mol⁻¹
	若非註明，所有數據均出自標準狀態（25 ℃，100 kPa）下。

甲基丙二酸單醯輔酶A （或簡稱甲基丙二醯輔酶A）是由輔酶A與甲基丙二酸通過硫酯鍵結合的重要代謝中間產物，參與許多生物合成與分解反應。^[1]

生物合成及轉換

由奇數碳的脂肪酸代謝或膽固醇側鏈形成的丙醯輔酶A與碳酸氫鹽經丙醯輔酶A羧化酶催化形成甲基丙二醯輔酶A^[2]^[3]。甲基丙二醯輔酶A再通過甲基丙二酸單醯輔酶A變位酶（methylmalonyl-CoA mutase，MUT）異構化為琥珀醯輔酶A（以維生素B₁₂為輔酶）。此異構化的反應是可逆的，其產物琥珀醯輔酶A進入三羧酸循環^[4]。即:

丙醯輔酶A + 碳酸氫鹽 → 甲基丙二醯輔酶A → 琥珀醯輔酶A

維生素B₁₂

維生素B₁₂在此反應中起重要作用。輔酶B₁₂（腺苷鈷胺素）是維生素B₁₂的一種有機金屬形式。它是甲基丙二醯輔酶A變位酶的輔因子^[5]。甲基丙二醯輔酶A轉化為琥珀醯輔酶A是自由基反應。

另見

甲基丙二酸單醯輔酶A差向異構酶

參考文獻

^ 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. （原始內容存檔於2022-10-11）（英語）.
^ ^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|title=為空 (幫助)
^ Kräutler, Bernhard. Biochemistry of B12-Cofactors in Human Metabolism. Stanger, Olaf (編). 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 （英語）.
^ ^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 （英語）.
^ 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. （原始內容存檔於2022-03-19）（英語）.

[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 . PMID 29033250. doi:10.1016/j.ymgme.2017.10.002. （原始內容存檔於2022-10-11）（英語）.

[: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.

[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=為空 (幫助)

[5] Kräutler, Bernhard. Biochemistry of B12-Cofactors in Human Metabolism. Stanger, Olaf (編). 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 （英語）.

[: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 （英語）.

[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. （原始內容存檔於2022-03-19）（英語）.

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甲基丙二酸單醯輔酶A

生物合成及轉換

維生素B₁₂

相關疾病

甲基丙二酸血症（Methylmalonic Acidemia，MMA）

另見

參考文獻

生物合成及轉換

維生素B12

相關疾病

甲基丙二酸血症（Methylmalonic Acidemia，MMA）

另見

參考文獻

維生素B₁₂