HIGD2A is Required for Assembly of the COX3 Module of Human Mitochondrial Complex IV.

ABSTRACT: Assembly factors play a critical role in the biogenesis of mitochondrial respiratory chain complexes I-IV where they assist in the membrane insertion of subunits, attachment of co-factors, and stabilization of assembly intermediates. The major fraction of complexes I, III and IV are present together in large molecular structures known as respiratory chain supercomplexes. Several assembly factors have been proposed as required for supercomplex assembly, including the hypoxia inducible gene 1 domain family member HIGD2A. Using gene-edited human cell lines and extensive steady state, translation and affinity enrichment proteomics techniques we show that loss of HIGD2A leads to defects in the de novo biogenesis of mtDNA-encoded COX3, subsequent accumulation of complex IV intermed

SUBMITTER: Hock DH 

PROVIDER: S-EPMC7338084 | biostudies-literature | 2020 Jul

REPOSITORIES: biostudies-literature