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
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!
MCCC2 mutants [mitochondrial matrix]
Stable Identifier
R-HSA-9914221
Type
Set [DefinedSet]
Species
Homo sapiens
Compartment
mitochondrial matrix
Locations in the PathwayBrowser
Expand all
Disease (Homo sapiens)
Diseases of metabolism (Homo sapiens)
Diseases of branched-chain amino acid catabolism (Homo sapiens)
3-Methylcrotonyl-CoA carboxylase deficiency (Homo sapiens)
MCCC mutants don't synthesize beta-methylglutaconyl-CoA (Homo sapiens)
MCCC mutant complexes [mitochondrial matrix] (Homo sapiens)
6x(Btn-MCCC1:MCCC2 mutants) [mitochondrial matrix] (Homo sapiens)
MCCC2 mutants [mitochondrial matrix] (Homo sapiens)
Participants
members
A456V MCCC2 [mitochondrial matrix]
(Homo sapiens)
E99Q MCCC2 [mitochondrial matrix]
(Homo sapiens)
G352R MCCC2 [mitochondrial matrix]
(Homo sapiens)
H190Y MCCC2 [mitochondrial matrix]
(Homo sapiens)
S173L MCCC2 [mitochondrial matrix]
(Homo sapiens)
V339M MCCC2 [mitochondrial matrix]
(Homo sapiens)
Participates
as a component of
6x(Btn-MCCC1:MCCC2 mutants) [mitochondrial matrix] (Homo sapiens)
Disease
Name
Identifier
Synonyms
3-methylcrotonyl-CoA carboxylase deficiency
DOID:0050710
3MCC deficiency, BMCC deficiency, 3-Methylcrotonylglycinuria
3-Methylcrotonyl-CoA carboxylase 2 deficiency
DOID:0080580
© 2025
Reactome
Cite Us!
Cite Us!
Cite Us!
Warning!
Unable to extract citation. Please try again later.
Download As:
BibTeX
RIS
Text