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Details on Person UniProt:Q8IUX1 TMEM126B

Class:IdReferenceGeneProduct:250200
_chainChangeLogchain:1-230 added on Sat February 7 2015;chain:1-230 removed on Tue November 3 2015;initiator methionine:1 added on Tue November 3 2015;chain:2-230 added on Tue November 3 2015;initiator methionine:1 for 250200 removed on Fri Nov 03 2023;initiator methionine: for 250200 added on Fri Nov 03 2023;initiator methionine: for 250200 removed on Fri Aug 15 2025;initiator methionine:1 for 250200 added on Fri Aug 15 2025
_displayNameUniProt:Q8IUX1 TMEM126B
_timestamp2025-08-15 21:49:30
chaininitiator methionine:1
chain:2-230
checksumB0EB374211C3CF52
commentFUNCTION As part of the MCIA complex, involved in the assembly of the mitochondrial complex I (PubMed:27374773, PubMed:27374774, PubMed:32320651). Participates in constructing the membrane arm of complex I (PubMed:24191001).SUBUNIT Part of the mitochondrial complex I assembly/MCIA complex that comprises at least the core subunits TMEM126B, NDUFAF1, ECSIT and ACAD9 and complement subunits such as COA1 and TMEM186 (PubMed:32320651). Associates with the intermediate 370 kDa subcomplex of incompletely assembled complex I (PubMed:24191001). Interacts with TMEM70 (PubMed:33753518).INTERACTION The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the TMEM126 family.
created[InstanceEdit:217385] Schmidt, EE, 2008-03-27 06:23:53
descriptionrecommendedName: fullName evidence="11"Complex I assembly factor TMEM126B, mitochondrial alternativeName: Transmembrane protein 126B
geneNameTMEM126B
HT007
identifierQ8IUX1
isSequenceChangedFALSE
keywordAlternative splicing
Chaperone
Disease variant
Membrane
Mitochondrion
Phosphoprotein
Primary mitochondrial disease
Proteomics identification
Reference proteome
Transmembrane
Transmembrane helix
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9917590] Weiser, Joel, 2024-08-09
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9963647] Weiser, Joel, 2025-08-15
nameTMEM126B
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8993347] ENSEMBL:ENSG00000171204 TMEM126B [Homo sapiens]
secondaryIdentifierT126B_HUMAN
A8K535
A8MSS0
Q32Q09
Q8WVU3
Q96EP3
Q9NZ29
sequenceLength230
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:250201] UniProt:Q8IUX1-2 TMEM126B [Homo sapiens]
[ReferenceIsoform:250202] UniProt:Q8IUX1-3 TMEM126B [Homo sapiens]
[ReferenceIsoform:250203] UniProt:Q8IUX1-4 TMEM126B [Homo sapiens]
[ReferenceIsoform:250204] UniProt:Q8IUX1-5 TMEM126B [Homo sapiens]
[ReferenceIsoform:410792] UniProt:Q8IUX1-1 TMEM126B [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:5689046] TMEM126B [mitochondrial inner membrane] [Homo sapiens]
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