FEATURES

FAMILY

DESCRIPTION

Also known as T126B_HUMAN, TMEM126B. As part of the MCIA complex, involved in the assembly of the mitochondrial complex I (PubMed:32320651, PubMed:27374773, PubMed:27374774). Participates in constructing the membrane arm of complex I (PubMed:24191001). 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).

EXTERNAL LINKS

uniprot
enzyme classification
PhosphoSitePlus

TMEM126B

Complex I assembly factor TMEM126B, mitochondrial

TMEM126B

Molecular Synopsis