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Details on Person beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA)
Class:Id Reaction:70773
_displayName beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA)
_doRelease TRUE
_timestamp 2024-12-03 20:53:17
catalystActivity [CatalystActivity:70771] methylcrotonoyl-CoA carboxylase activity of 6x(Btn-MCCC1:MCCC2) [mitochondrial matrix]
catalystActivityReference [CatalystActivityReference:9643717] methylcrotonoyl-CoA carboxylase activity of 6x(Btn-MCCC1:MCCC2) [mitochondrial matrix] The molecular basis of human 3-methylcrotonyl-CoA carboxylase deficiency
compartment [Compartment:5460] mitochondrial matrix
crossReference [DatabaseIdentifier:9925947] RHEA:13590
input [SimpleEntity:70744] bMC-CoA [mitochondrial matrix] [SimpleEntity:113593] ATP [mitochondrial matrix] [SimpleEntity:29896] HCO3- [mitochondrial matrix]
internalReviewed [InstanceEdit:9917225] D'Eustachio, Peter, 2024-08-01
literatureReference [LiteratureReference:70761] The molecular basis of human 3-methylcrotonyl-CoA carboxylase deficiency [LiteratureReference:1112670] Subcellular localization of 3-methylcrotonyl-coenzyme A carboxylase in bovine kidney [LiteratureReference:70749] Cloning of the human MCCA and MCCB genes and mutations therein reveal the molecular cause of 3-methylcrotonyl-CoA: carboxylase deficiency [LiteratureReference:1112677] Mitochondrial targeting signals and mature peptides of 3-methylcrotonyl-CoA carboxylase
modified [InstanceEdit:74479] D'Eustachio, P, 2003-07-22 16:21:00 [InstanceEdit:113517] Schmidt, EE, 2004-06-09 05:19:12 [InstanceEdit:163376] Vastrik, I, 2005-04-29 06:26:51 [InstanceEdit:508306] D'Eustachio, P, 2010-02-11 [InstanceEdit:517476] D'Eustachio, P, 2010-02-18 [InstanceEdit:580603] D'Eustachio, P, 2010-04-11 [InstanceEdit:1112674] D'Eustachio, P, 2010-12-08 [InstanceEdit:8946993] Shorser, Solomon, 2016-11-07 [InstanceEdit:8981172] Duenas, Corina, 2017-03-03 [InstanceEdit:9643047] Wu, G, 2019-04-04 [InstanceEdit:9830342] Matthews, Lisa, 2023-03-08 [InstanceEdit:9907327] Stephan, Ralf, 2024-04-06 [InstanceEdit:9908873] Stephan, Ralf, 2024-04-23 [InstanceEdit:9917131] Stephan, Ralf, 2024-07-30 [InstanceEdit:9919308] D'Eustachio, Peter, 2024-08-20 [InstanceEdit:9919317] D'Eustachio, Peter, 2024-08-21 [InstanceEdit:9925948] D'Eustachio, Peter, 2024-10-28 [InstanceEdit:9930378] D'Eustachio, Peter, 2024-12-03
name beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA) 3-methyl-(2E)-butenoyl-CoA + ATP + hydrogencarbonate => 3-methyl-(2E)-glutaconyl-CoA + ADP + H(+) + phosphate
negativePrecedingEvent [NegativePrecedingEvent:9908863] Futile Cycle: beta-methylglutaconyl-CoA + ADP + orthophosphate <=> beta-methylcrotonyl-CoA + ATP + HCO3- (MCCA) [NegativePrecedingEvent:9930391] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+
output [SimpleEntity:70772] bMG-CoA [mitochondrial matrix] [SimpleEntity:113581] ADP [mitochondrial matrix] [SimpleEntity:113548] Pi [mitochondrial matrix] [SimpleEntity:113529] H+ [mitochondrial matrix]
precedingEvent
previousReviewStatus [ReviewStatus:9821385] four stars
releaseDate 2004-07-06
reverseReaction
reviewed [InstanceEdit:9917333] Hill, David P, 2024-08-02
reviewStatus [ReviewStatus:9821382] five stars
species [Species:48887] Homo sapiens
stableIdentifier [StableIdentifier:359855] R-HSA-70773.9
structureModified [InstanceEdit:9917131] Stephan, Ralf, 2024-07-30
summation [Summation:178506] Methylcrotonyl CoA carboxylase (MCCA) catalyzes the reversib...
(hasEvent) [Pathway:70895] Branched-chain amino acid catabolism [Homo sapiens]
(normalReaction) [FailedReaction:9909466] MCCC mutants don't synthesize beta-methylglutaconyl-CoA [Homo sapiens]
(precedingEvent) [Reaction:70785] beta-methylglutaconyl-CoA + H2O <=> beta-hydroxy-beta-methylglutaryl-CoA [Homo sapiens] [NegativePrecedingEvent:9908866] Futile Cycle: beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA)
(reverseReaction) [Reaction:508308] beta-methylglutaconyl-CoA + ADP + orthophosphate <=> beta-methylcrotonyl-CoA + ATP + HCO3- (MCCA) [Homo sapiens]
(updatedInstance) [_UpdateTracker:9789910] Update Tracker - [Reaction:70773] beta-methylcrotonyl-CoA + ATP + CO2 <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O [MCCA] - v36:[addLiteratureReference, modifyText] [_UpdateTracker:9790396] Update Tracker - [Reaction:70773] beta-methylcrotonyl-CoA + ATP + CO2 <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O [MCCA] - v33:[add_removeInput, removeOutput] [_UpdateTracker:9791078] Update Tracker - [Reaction:70773] beta-methylcrotonyl-CoA + ATP + CO2 <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O [MCCA] - v32:[modifyText] [_UpdateTracker:9919348] Update Tracker - [Reaction:70773] beta-methylcrotonyl-CoA + ATP + CO2 <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O [MCCA] - v90:[modifyText, removeOutput]
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No pathways have been reviewed or authored by beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA) (70773)