Reactome Pathway Database
Toggle navigation
About
What is Reactome ?
News
Team
Scientific Advisory Board
Funding
Editorial Calendar
Release Calendar
Statistics
Our Logo
License Agreement
Privacy Notice
Disclaimer
Digital Preservation
Contact us
Content
Table of Contents
DOIs
Data Schema
Reactome Research Spotlight
ORCID Integration Project
COVID-19 Disease Pathways
Docs
Userguide
Pathway Browser
How do I search ?
Details Panel
Analysis Tools
Analysis Data
Analysis Gene Expression
Species Comparison
Tissue Distribution
Diseases
Cytomics
Review Status of Reactome Events
ReactomeFIViz
Developer's Zone
Graph Database
Analysis Service
Content Service
Pathways Overview
Pathway Diagrams
Icon Info
EHLD Specs & Guidelines
Icon Library Guidelines
Data Model
Curator Guide
Release Documentation
Computationally inferred events
FAQ
Linking to Us
Citing us
Tools
Pathway Browser
Analyse gene list
Analyse gene expression
Species Comparison
Tissue Distribution
Analysis Service
Content Service
ReactomeFIViz
Advanced Data Search
Site Search
Community
Contribute Pathway Knowledge
Icon Library
Outreach
Events
Publications
Partners
Contributors
Resources Guide
Download
Reactome Pathway Database
About
What is Reactome ?
News
Team
Scientific Advisory Board
Funding
Editorial Calendar
Release Calendar
Statistics
Our Logo
License Agreement
Privacy Notice
Disclaimer
Digital Preservation
Contact us
Content
Table of Contents
DOIs
Data Schema
Reactome Research Spotlight
ORCID Integration Project
COVID-19 Disease Pathways
Docs
Userguide
Pathway Browser
How do I search ?
Details Panel
Analysis Tools
Analysis Data
Analysis Gene Expression
Species Comparison
Tissue Distribution
Diseases
Cytomics
Review Status of Reactome Events
ReactomeFIViz
Developer's Zone
Graph Database
Analysis Service
Content Service
Pathways Overview
Pathway Diagrams
Icon Info
EHLD Specs & Guidelines
Icon Library Guidelines
Data Model
Curator Guide
Release Documentation
Computationally inferred events
FAQ
Linking to Us
Citing us
Tools
Pathway Browser
Analyse gene list
Analyse gene expression
Species Comparison
Tissue Distribution
Analysis Service
Content Service
ReactomeFIViz
Advanced Data Search
Site Search
Community
Contribute Pathway Knowledge
Icon Library
Outreach
Events
Publications
Partners
Contributors
Resources Guide
Download
Search ...
Go!
Schema
>
Reaction
>
Entries
beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA)
Show undefined attributes
Go to Details
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
category
transition
compartment
[Compartment:5460] mitochondrial matrix
crossReference
[DatabaseIdentifier:12610808] Rhea:13590
[DatabaseIdentifier:9925947] RHEA:13590
dbId
70773
displayName
beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA)
eventOf
[Pathway:R-HSA-70895] Branched-chain amino acid catabolism - Homo sapiens
followingEvent
[Reaction:R-HSA-70785] beta-methylglutaconyl-CoA + H2O <=> beta-hydroxy-beta-methylglutaryl-CoA - Homo sapiens
input
[SimpleEntity:R-ALL-113593] ATP [mitochondrial matrix]
[SimpleEntity:R-ALL-70744] bMC-CoA [mitochondrial matrix]
[SimpleEntity:R-ALL-29896] HCO3- [mitochondrial matrix]
isInDisease
false
isInferred
false
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
maxDepth
1
modified
[InstanceEdit:10948619] Weiser, Joel, 2025年08月27日
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+
orthologousEvent
[Reaction:R-MMU-70773] beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA) - Mus musculus
[Reaction:R-DDI-70773] beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA) - Dictyostelium discoideum
[Reaction:R-CEL-70773] beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA) - Caenorhabditis elegans
[Reaction:R-DME-70773] beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA) - Drosophila melanogaster
[Reaction:R-GGA-70773] beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA) - Gallus gallus
[Reaction:R-XTR-70773] beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA) - Xenopus tropicalis
[Reaction:R-SSC-70773] beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA) - Sus scrofa
[Reaction:R-BTA-70773] beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA) - Bos taurus
[Reaction:R-CFA-70773] beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA) - Canis familiaris
[Reaction:R-RNO-70773] beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA) - Rattus norvegicus
output
[SimpleEntity:R-ALL-113581] ADP [mitochondrial matrix]
[SimpleEntity:R-ALL-70772] bMG-CoA [mitochondrial matrix]
[SimpleEntity:R-ALL-113529] H+ [mitochondrial matrix]
[SimpleEntity:R-ALL-113548] Pi [mitochondrial matrix]
precedingEvent
[Reaction:R-HSA-70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - Homo sapiens
previousReviewStatus
[ReviewStatus:9821385] four stars
releaseDate
2004年07月06日
reviewStatus
[ReviewStatus:9821382] five stars
reviewed
[InstanceEdit:9917333] Hill, David P, 2024年08月02日
schemaClass
Reaction
species
[Species:48887] Homo sapiens
stId
R-HSA-70773
summation
[Summation:178506] Methylcrotonyl CoA carboxylase (MCCA) catalyzes the reversib...
updateTrackers
[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]
[UpdateTracker:9790396] Update Tracker - [Reaction:70773] beta-methylcrotonyl-CoA + ATP + CO2 <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O [MCCA] - v33:[add_removeInput, removeOutput]
[UpdateTracker:9789910] Update Tracker - [Reaction:70773] beta-methylcrotonyl-CoA + ATP + CO2 <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O [MCCA] - v36:[addLiteratureReference, modifyText]
Referrals
(updatedInstance)
[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]
[UpdateTracker:9790396] Update Tracker - [Reaction:70773] beta-methylcrotonyl-CoA + ATP + CO2 <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O [MCCA] - v33:[add_removeInput, removeOutput]
[UpdateTracker:9789910] Update Tracker - [Reaction:70773] beta-methylcrotonyl-CoA + ATP + CO2 <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O [MCCA] - v36:[addLiteratureReference, modifyText]
(normalReaction)
[FailedReaction:R-HSA-9909466] MCCC mutants don't synthesize beta-methylglutaconyl-CoA
(hasEvent)
[Pathway:R-HSA-70895] Branched-chain amino acid catabolism
(precedingEvent)
[Reaction:R-HSA-70785] beta-methylglutaconyl-CoA + H2O <=> beta-hydroxy-beta-methylglutaryl-CoA
[NegativePrecedingEvent:9908866] Futile Cycle: beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA)
(reverseReaction)
[Reaction:R-HSA-508308] beta-methylglutaconyl-CoA + ADP + orthophosphate <=> beta-methylcrotonyl-CoA + ATP + HCO3- (MCCA)
© 2025
Reactome
Cite Us!
Cite Us!
Cite Us!
Warning!
Unable to extract citation. Please try again later.
Download As:
BibTeX
RIS
Text
Download
Close
AltStyle
によって変換されたページ
(->オリジナル)
/
アドレス:
モード:
デフォルト
音声ブラウザ
ルビ付き
配色反転
文字拡大
モバイル