A dual function of TMEM70 in OXPHOS: assembly of complexes I and V

• DOI: 10.1101/697185
• Publication Date: 2019-07-10T22:25:21Z
• Authors: Laura Sánchez-Cab...; Dei M. Elurbe; Fabian Baertling; Sergio Guerrero-C...; Mariel van den Brand; Joeri van Strien; Teunis J. P. van Dam; Richard Rodenburg; Ulrich Brandt; Martijn A. Huynen; Leo G.J. Nijtmans
• Keywords: Protein Stability

Abstract Protein complexes from the oxidative phosphorylation (OXPHOS) system are assembled with help of proteins called assembly factors. We here delineate function inner mitochondrial membrane protein TMEM70, in which mutations have been linked to OXPHOS deficiencies, using a combination BioID, complexome profiling and coevolution analyses. TMEM70 interacts complex I V for both loss results accumulation an intermediate followed by reduction next pathway. This indicates that has role stability membrane-bound subassemblies or recruitment subunits into forming complex. Independent evidence comes evolutionary The TMEM70/TMEM186/TMEM223 family, we show TMEM186 TMEM223 human as well, only occurs species complexes. Our validate use combining complexomics BioID analyses elucidating congenital defects assembly.