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!
BCAA-lipoH2-K105-DBT [mitochondrial matrix]
Stable Identifier
R-HSA-9859149
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)
Maple Syrup Urine Disease (Homo sapiens)
Loss-of-function mutations in DBT cause MSUD2 (Homo sapiens)
DBT loss-of-function mutants don't synthesize BCAA-CoA (Homo sapiens)
BCAA-lipoH2-K105-DBT [mitochondrial matrix] (Homo sapiens)
Metabolism (Homo sapiens)
Metabolism of amino acids and derivatives (Homo sapiens)
Branched-chain amino acid catabolism (Homo sapiens)
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV (Homo sapiens)
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV (Homo sapiens)
BCAA-lipoH2-K105-DBT [mitochondrial matrix] (Homo sapiens)
DBT transfers BCAA to CoA (Homo sapiens)
BCAA-lipoH2-K105-DBT [mitochondrial matrix] (Homo sapiens)
Participants
members
2-Methylbutyryl-lipoH2-K105-DBT [mitochondrial matrix]
(Homo sapiens)
Isobutyryl-lipoH2-K105-DBT [mitochondrial matrix]
(Homo sapiens)
Isovaleryl-lipoH2-K105-DBT [mitochondrial matrix]
(Homo sapiens)
Participates
as an input of
DBT loss-of-function mutants don't synthesize BCAA-CoA (Homo sapiens)
DBT transfers BCAA to CoA (Homo sapiens)
as an output of
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV (Homo sapiens)
Inferred To
Dbt [mitochondrial matrix]
(Rattus norvegicus)
BCAA-lipoH2-K105-DBT [mitochondrial matrix]
(Bos taurus)
BCAA-lipoH2-K105-DBT [mitochondrial matrix]
(Caenorhabditis elegans)
BCAA-lipoH2-K105-DBT [mitochondrial matrix]
(Gallus gallus)
BCAA-lipoH2-K105-DBT [mitochondrial matrix]
(Mus musculus)
BCAA-lipoH2-K105-DBT [mitochondrial matrix]
(Plasmodium falciparum)
dbt [mitochondrial matrix]
(Danio rerio)
dbt [mitochondrial matrix]
(Xenopus tropicalis)
Dbct [mitochondrial matrix]
(Drosophila melanogaster)
bkdC [mitochondrial matrix]
(Dictyostelium discoideum)
DBT [mitochondrial matrix]
(Canis familiaris)
DBT [mitochondrial matrix]
(Sus scrofa)
© 2025
Reactome
Cite Us!
Cite Us!
Cite Us!
Warning!
Unable to extract citation. Please try again later.
Download As:
BibTeX
RIS
Text