| Revision as of 11:14, 27 May 2011 editEdgar181 (talk | contribs)Extended confirmed users196,325 edits minor cleanup← Previous edit |
Latest revision as of 20:34, 15 September 2024 edit undoCitation bot (talk | contribs)Bots5,459,817 edits Added title. Removed parameters. Some additions/deletions were parameter name changes. | Use this bot. Report bugs. | Suggested by Whoop whoop pull up | Category:Thioesters of coenzyme A | #UCB_Category 24/35 |
| (39 intermediate revisions by 24 users not shown) |
| Line 1: |
Line 1: |
|
{{Chembox |
|
{{Chembox |
|
|
| Verifiedfields = changed |
| ⚫ |
| verifiedrevid = 413362156 |
|
|
|
| Watchedfields = changed |
| ⚫ |
| ImageFile = Methylmalonyl-CoA.PNG |
|
|
⚫ |
| verifiedrevid = 431161699 |
|
⚫ |
| ImageFile = Methylmalonyl-CoA.svg |
|
| ImageSize = 200px |
|
| ImageSize = 200px |
|
|
| SystematicName = (9''R'')-1--3,5,9-trihydroxy-8,8,20-trimethyl-3,5,10,14,19-pentaoxo-2,4,6-trioxa-18-thia-11,15-diaza-3λ<sup>5</sup>,5λ<sup>5</sup>-diphosphahenicosan-21-oic acid |
|
| IUPACName = |
|
|
| OtherNames = |
|
| OtherNames = |
|
| Section1 = {{Chembox Identifiers |
|
|Section1={{Chembox Identifiers |
|
|
| IUPHAR_ligand = 5223 |
|
| CASNo = 1264-45-5 |
|
|
|
| CASNo_Ref = {{cascite|correct|??}} |
| ⚫ |
| PubChem = 123909 |
|
|
| SMILES = |
|
| CASNo = 1264-45-5 |
|
⚫ |
| PubChem = 123909 |
|
| MeSHName = methylmalonyl-coenzyme+A |
|
|
|
| ChemSpiderID_Ref = {{chemspidercite|changed|chemspider}} |
|
|
| ChemSpiderID = 110440| ChEBI_Ref = {{ebicite|changed|EBI}} |
|
|
| ChEBI = 16625 |
|
|
| SMILES = CC(C(=O)O)C(=O)SCCNC(=O)CCNC(=O)(C(C)(C)COP(=O)(O)OP(=O)(O)OC1(((O1)N2C=NC3=C(N=CN=C32)N)O)OP(=O)(O)O)O |
|
|
| 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 |
|
|
| StdInChI_Ref = {{stdinchicite|changed|chemspider}} |
|
|
| StdInChI = 1S/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 |
|
|
| StdInChIKey_Ref = {{stdinchicite|changed|chemspider}} |
|
|
| StdInChIKey = MZFOKIKEPGUZEN-FBMOWMAESA-N |
|
}} |
|
}} |
|
| Section2 = {{Chembox Properties |
|
|Section2={{Chembox Properties |
|
| Formula = C<sub>25</sub>H<sub>40</sub>N<sub>7</sub>O<sub>19</sub>P<sub>3</sub>S |
|
| Formula = C<sub>25</sub>H<sub>40</sub>N<sub>7</sub>O<sub>19</sub>P<sub>3</sub>S |
|
| MolarMass = 867.608 g/mol |
|
| MolarMass = 867.608 g/mol |
|
| Appearance = |
|
| Appearance = |
|
| Density = |
|
| Density = |
|
| MeltingPt = |
|
| MeltingPt = |
|
| BoilingPt = |
|
| BoilingPt = |
|
|
| Solubility = |
|
}} |
|
}} |
|
| Section3 = {{Chembox Hazards |
|
|Section3={{Chembox Hazards |
|
| Solubility = |
|
| MainHazards = |
|
| MainHazards = |
|
| FlashPt = |
|
| FlashPt = |
|
| AutoignitionPt = |
|
| Autoignition = |
|
|
}} |
|
}} |
|
|
}}'''Methylmalonyl-CoA''' is the ] consisting of ] linked to ]. It is an important intermediate in the ] of ], which plays an essential role in the tricarboxylic acid cycle (aka the ]).<ref name="Wongkittichote_2017">{{cite journal | vauthors = Wongkittichote P, Ah Mew N, Chapman KA | title = Propionyl-CoA carboxylase - A review | journal = Molecular Genetics and Metabolism | volume = 122 | issue = 4 | pages = 145–152 | date = December 2017 | pmid = 29033250 | pmc = 5725275 | doi = 10.1016/j.ymgme.2017.10.002 }}</ref> |
|
}} |
|
|
|
|
|
|
|
== Biosynthesis and metabolism == |
|
'''Methylmalonyl-CoA''' is the ] linked form of ]. Methylmalonyl-CoA is formed from ] by ] by help of ] (vitamin B<sub>7</sub>). It is converted into ] by ], in a reaction that requires ] as a cofactor. In this way, it enters the ], and is thus part of one of the ]. The following diagram demonstrates the aforementioned reactions: |
|
|
|
] |
|
|
Methylmalonyl-CoA results from the ] of ] with an ] of ], of ] ], ], ], ] or of ], forming ].<ref name="Baumgartner_2014">{{cite journal | vauthors = Baumgartner MR, Hörster F, Dionisi-Vici C, Haliloglu G, Karall D, Chapman KA, Huemer M, Hochuli M, Assoun M, Ballhausen D, Burlina A, Fowler B, Grünert SC, Grünewald S, Honzik T, Merinero B, Pérez-Cerdá C, Scholl-Bürgi S, Skovby F, Wijburg F, MacDonald A, Martinelli D, Sass JO, Valayannopoulos V, Chakrapani A | display-authors = 6 | title = Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia | journal = Orphanet Journal of Rare Diseases | volume = 9 | issue = 1 | pages = 130 | date = September 2014 | pmid = 25205257 | pmc = 4180313 | doi = 10.1186/s13023-014-0130-8 | doi-access = free }}</ref> The latter is also formed from ], which bacteria produce in the intestine.<ref name="Baumgartner_2014" /> Propionyl-CoA and ] are converted to Methylmalonyl-CoA by the enzyme ].<ref name="Wongkittichote_2017" /> It then is converted into succinyl-CoA by ] (MUT). This reaction is a ] ]. In this way, the compound enters the Citric Acid Cycle. The following diagram demonstrates the aforementioned reaction:<ref name="Lehninger4th">{{Lehninger4th||name-list-style=vanc}}</ref> |
|
|
|
|
|
|
Propionyl CoA + Bicarbonate <big>→</big> Methylmalonyl CoA <big>→</big> Succinyl CoA |
|
:] → Methylmalonyl CoA → ] <ref>Hoffbrand,A.V., Moss, P.A.H, Pettit, J.E. ''Essential Haematology''. Blackwell publishing. 5th Ed. 2006. p. 46 </ref> |
|
|
|
|
|
|
|
== Vitamin B<sub>12</sub> == |
|
==See also== |
|
|
|
] plays an integral role in this reaction. Coenzyme B<sub>12</sub> (]) is an ] form of ] and serves as the ] of Methylmalonyl-CoA mutase, which is an essential ] in the human body.<ref name="Kräutler_2012">{{cite book | vauthors = Kräutler B |chapter=Biochemistry of B12-cofactors in human metabolism |date=2012 |title=Water Soluble Vitamins |series=Subcellular Biochemistry |volume=56 |pages=323–346 | veditors = Stanger O |place=Dordrecht |publisher=Springer Netherlands |doi=10.1007/978-94-007-2199-9_17 |pmid=22116707 |isbn=978-94-007-2198-2 }}</ref> The transformation of Methylmalonyl-CoA to Succinyl-CoA by this enzyme is a ].<ref name="Kräutler_2012" /> |
|
* ] |
|
|
|
== Related diseases == |
|
|
|
|
|
|
=== Methylmalonic Acidemia (MMA) === |
| ⚫ |
==References== |
|
|
|
{{main|Methylmalonyl-CoA mutase deficiency}} |
|
{{reflist}} |
|
|
|
This disease occurs when methylmalonyl-CoA mutase is unable to isomerize sufficient amounts of methylmalonyl-CoA into succinyl-CoA.<ref name="Takahashi-Iñiguez_2012">{{cite journal | vauthors = Takahashi-Iñiguez T, García-Hernandez E, Arreguín-Espinosa R, Flores ME | title = Role of vitamin B12 on methylmalonyl-CoA mutase activity | journal = Journal of Zhejiang University. Science. B | volume = 13 | issue = 6 | pages = 423–437 | date = June 2012 | pmid = 22661206 | pmc = 3370288 | doi = 10.1631/jzus.B1100329 }}</ref> This causes a buildup of propionic and/or methylmalonic acid, which has effects on infants ranging from severe brain damage to death.<ref name="Baumgartner_2014" /> The disease is linked to Vitamin B<sub>12</sub>, which is a cofactor for the enzyme methylmalonyl-CoA mutase.<ref name="Takahashi-Iñiguez_2012" /><ref name="Froese_2019">{{cite journal |vauthors=Froese DS, Fowler B, Baumgartner MR |date=July 2019 |title=Vitamin B<sub>12</sub> , folate, and the methionine remethylation cycle-biochemistry, pathways, and regulation |journal=Journal of Inherited Metabolic Disease |volume=42 |issue=4 |pages=673–685 |doi=10.1002/jimd.12009 |pmid=30693532 |doi-access=free}}</ref> |
|
|
|
|
|
|
=== Combined malonic and methylmalonic aciduria (CMAMMA) === |
|
|
In the metabolic disease ] (CMAMMA), ] (ACSF3) is reduced, which converts toxic methylmalonic acid to methylmalonyl-CoA and thus supplies it to the citric acid cycle.<ref>{{cite journal | vauthors = Gabriel MC, Rice SM, Sloan JL, Mossayebi MH, Venditti CP, Al-Kouatly HB | title = Considerations of expanded carrier screening: Lessons learned from combined malonic and methylmalonic aciduria | journal = Molecular Genetics & Genomic Medicine | volume = 9 | issue = 4 | pages = e1621 | date = April 2021 | pmid = 33625768 | pmc = 8123733 | doi = 10.1002/mgg3.1621 }}</ref><ref>{{cite journal | vauthors = Bowman CE, Wolfgang MJ | title = Role of the malonyl-CoA synthetase ACSF3 in mitochondrial metabolism | journal = Advances in Biological Regulation | volume = 71 | pages = 34–40 | date = January 2019 | pmid = 30201289 | pmc = 6347522 | doi = 10.1016/j.jbior.2018.09.002 }}</ref> The result is an accumulation of methylmalonic acid. |
|
|
|
|
⚫ |
== References == |
|
|
{{Reflist}} |
|
|
|
|
|
{{Amino acid metabolism intermediates}} |
|
{{Amino acid metabolism intermediates}} |
|
|
|
|
|
{{DEFAULTSORT:Methylmalonyl-Coa}} |
|
{{DEFAULTSORT:Methylmalonyl-Coa}} |
|
] |
|
] |
|
|
|
|
|
|
|
|
{{Microbiology-stub}} |
|
{{Microbiology-stub}} |
|
|
|
|
] |
|
|
] |
|
|
] |
|