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!
TMEM126B [mitochondrial inner membrane]
Stable Identifier
R-HSA-5689046
Type
Protein [EntityWithAccessionedSequence]
Species
Homo sapiens
Compartment
mitochondrial inner membrane
Synonyms
Complex I assembly factor TMEM126B, mitochondrial, T126B_HUMAN
Locations in the PathwayBrowser
Expand all
Metabolism (Homo sapiens)
Aerobic respiration and respiratory electron transport (Homo sapiens)
Respiratory electron transport (Homo sapiens)
Complex I biogenesis (Homo sapiens)
COA1:MT-ND2, TMEM186:MT-ND3, MT-ND6, NDUFB6 bind the MCIA complex to form a 370kDa subcomplex (Homo sapiens)
370 kDa subcomplex [mitochondrial inner membrane] (Homo sapiens)
MCIA complex [mitochondrial inner membrane] (Homo sapiens)
TMEM126B [mitochondrial inner membrane] (Homo sapiens)
MCIA complex [mitochondrial inner membrane] (Homo sapiens)
TMEM126B [mitochondrial inner membrane] (Homo sapiens)
ND4, ND5 bind the 550kDa complex to form the 815kDa complex (Homo sapiens)
550kDa complex [mitochondrial inner membrane] (Homo sapiens)
370 kDa subcomplex [mitochondrial inner membrane] (Homo sapiens)
MCIA complex [mitochondrial inner membrane] (Homo sapiens)
TMEM126B [mitochondrial inner membrane] (Homo sapiens)
815kDa complex [mitochondrial inner membrane] (Homo sapiens)
550kDa complex [mitochondrial inner membrane] (Homo sapiens)
370 kDa subcomplex [mitochondrial inner membrane] (Homo sapiens)
MCIA complex [mitochondrial inner membrane] (Homo sapiens)
TMEM126B [mitochondrial inner membrane] (Homo sapiens)
Peripheral arm subunits bind the 815kDa complex to form a 980kDa complex (Homo sapiens)
815kDa complex [mitochondrial inner membrane] (Homo sapiens)
550kDa complex [mitochondrial inner membrane] (Homo sapiens)
370 kDa subcomplex [mitochondrial inner membrane] (Homo sapiens)
MCIA complex [mitochondrial inner membrane] (Homo sapiens)
TMEM126B [mitochondrial inner membrane] (Homo sapiens)
980kDa complex [mitochondrial inner membrane] (Homo sapiens)
815kDa complex [mitochondrial inner membrane] (Homo sapiens)
550kDa complex [mitochondrial inner membrane] (Homo sapiens)
370 kDa subcomplex [mitochondrial inner membrane] (Homo sapiens)
MCIA complex [mitochondrial inner membrane] (Homo sapiens)
TMEM126B [mitochondrial inner membrane] (Homo sapiens)
The 315kDa subcomplex binds the 370kDa subcomplex to form the 550kDa complex (Homo sapiens)
370 kDa subcomplex [mitochondrial inner membrane] (Homo sapiens)
MCIA complex [mitochondrial inner membrane] (Homo sapiens)
TMEM126B [mitochondrial inner membrane] (Homo sapiens)
550kDa complex [mitochondrial inner membrane] (Homo sapiens)
370 kDa subcomplex [mitochondrial inner membrane] (Homo sapiens)
MCIA complex [mitochondrial inner membrane] (Homo sapiens)
TMEM126B [mitochondrial inner membrane] (Homo sapiens)
The MCIA complex, NDUFAF2-7 all dissociate from the 980kDa complex, resulting in Complex I (Homo sapiens)
980kDa complex [mitochondrial inner membrane] (Homo sapiens)
815kDa complex [mitochondrial inner membrane] (Homo sapiens)
550kDa complex [mitochondrial inner membrane] (Homo sapiens)
370 kDa subcomplex [mitochondrial inner membrane] (Homo sapiens)
MCIA complex [mitochondrial inner membrane] (Homo sapiens)
TMEM126B [mitochondrial inner membrane] (Homo sapiens)
MCIA complex [mitochondrial inner membrane] (Homo sapiens)
TMEM126B [mitochondrial inner membrane] (Homo sapiens)
External Reference Information
External Reference
UniProt:Q8IUX1 TMEM126B
Gene Names
TMEM126B, HT007
Chain
initiator methionine:, chain:2-230
Reference Genes
BioGPS Gene:55863 TMEM126B
COSMIC (genes):TMEM126B TMEM126B
CTD Gene:55863 TMEM126B
dbSNP Gene:55863 TMEM126B
ENSEMBL:ENSG00000171204 TMEM126B
HGNC:30883 TMEM126B
KEGG:hsa:55863 TMEM126B
Monarch:55863 TMEM126B
NCBI Gene:55863 TMEM126B
OMIM:615533 TMEM126B
UCSC:Q8IUX1 TMEM126B
Reference Transcript
RefSeq:NM_018480.4 TMEM126B
RefSeq:XM_011545165.2 TMEM126B
RefSeq:NM_001193538.2 TMEM126B
RefSeq:NM_001256546.1 TMEM126B
Other Identifiers
11722392_a_at
11722393_s_at
11757380_s_at
11760713_at
1554537_PM_at
1554537_at
16729670
221622_PM_s_at
221622_s_at
3342984
3342986
3342987
3342988
3342989
3342990
3342991
3342992
3342993
3342994
3342995
3342996
3342997
3342998
3342999
3343000
45725_at
55863
7942879
A_14_P107133
A_23_P2129
GE85480
GO:0003674
GO:0005515
GO:0005739
GO:0005743
GO:0007005
GO:0016020
GO:0031966
GO:0032094
GO:0032981
GO:0043226
GO:0065003
HMNXSV003025240
Hs2.24371.2.S1_3p_at
ILMN_1766851
ILMN_2145518
PH_hs_0026457
PH_hs_0034023
TC11000864.hg
TC11002809.hg
g12005514_3p_a_at
Participates
as a component of
MCIA complex [mitochondrial inner membrane] (Homo sapiens)
Inferred To
Tmem126 [mitochondrial inner membrane]
(Drosophila melanogaster)
Ghost homologue of TMEM126B [mitochondrial inner membrane]
(Caenorhabditis elegans)
Tmem126b [mitochondrial inner membrane]
(Mus musculus)
Tmem126b [mitochondrial inner membrane]
(Rattus norvegicus)
tmem126a [mitochondrial inner membrane]
(Danio rerio)
tmem126a [mitochondrial inner membrane]
(Xenopus tropicalis)
TMEM126A [mitochondrial inner membrane]
(Gallus gallus)
TMEM126B [mitochondrial inner membrane]
(Bos taurus)
TMEM126B [mitochondrial inner membrane]
(Canis familiaris)
TMEM126B [mitochondrial inner membrane]
(Sus scrofa)
Cross References
ENSEMBL
ENST00000534341
,
ENST00000358867
,
ENSP00000433471
,
ENSG00000171204
,
ENSP00000377039
,
ENSP00000351737
,
ENST00000393375
OpenTargets
ENSG00000171204
GeneCards
TMEM126B
HPA
ENSG00000171204-TMEM126B
PRO
Q8IUX1
Pharos - Targets
Q8IUX1
Orphanet
TMEM126B
Interactors (3)
Accession
#Entities
Entities
Confidence Score
Evidence (IntAct)
UniProt:Q9BQ95 ECSIT
2
ECSIT [cytosol]
(R-HSA-168064)
ECSIT [mitochondrial inner membrane]
(R-HSA-5689047)
0.527
3
UniProt:Q9P0J0 NDUFA13
8
NDUFA13 [mitochondrial inner membrane]
(R-HSA-59936)
NDUFA13(41-49) [mitochondrial inner membrane]
(R-HSA-9839958)
NDUFA13(100-108) [mitochondrial inner membrane]
(R-HSA-9839959)
NDUFA13(109-144) [mitochondrial inner membrane]
(R-HSA-9839968)
NDUFA13(2-19) [mitochondrial inner membrane]
(R-HSA-9839966)
NDUFA13(69-99) [mitochondrial inner membrane]
(R-HSA-9839964)
NDUFA13(20-40) [mitochondrial inner membrane]
(R-HSA-9839963)
NDUFA13(50-68) [mitochondrial inner membrane]
(R-HSA-9839961)
0.527
3
UniProt:Q9NPL8 TIMMDC1
1
TIMMDC1 [mitochondrial inner membrane]
(R-HSA-5689720)
0.527
2
© 2025
Reactome
Cite Us!
Cite Us!
Cite Us!
Warning!
Unable to extract citation. Please try again later.
Download As:
BibTeX
RIS
Text