Five entry points of the mitochondrially encoded subunits in mammalian complex I assembly.
Complex I (CI) is the largest enzyme of the mammalian mitochondrial respiratory chain. The biogenesis of the complex is a very complex process due to its large size and number of subunits (45 subunits). The situation is further complicated due to the fact that its subunits have a double genomic origin, as seven of them are encoded by the mitochondrial DNA. Understanding of the assembly process and characterization of the involved factors has advanced very much in the last years. However, until now, a key part of the process, that is, how and at which step the mitochondrially encoded CI subunits (ND subunits) are incorporated in the CI assembly process, was not known. Analyses of several mouse cell lines mutated for three ND subunits allowed us to determine the importance of each one for complex assembly/stability and that there are five different steps within the assembly pathway in which some mitochondrially encoded CI subunit is incorporated.
Perales-Clemente
Ester
E
Centro Nacional de Investigaciones Cardiovasculares Carlos III, Melchor Fernández Almagro, 3, 28029 Madrid, Spain.
Fernández-Vizarra
Erika
E
Acín-Pérez
Rebeca
R
Movilla
Nieves
N
Bayona-Bafaluy
María Pilar
MP
Moreno-Loshuertos
Raquel
R
Pérez-Martos
Acisclo
A
Fernández-Silva
Patricio
P
Enríquez
José Antonio
JA
D000818:Animals; D001483:Base Sequence; D002460:Cell Line; D004252:DNA Mutational Analysis; D004272:DNA, Mitochondrial / Q000235:genetics*; D042967:Electron Transport Complex I / Q000235:genetics / Q000378:metabolism*; D004591:Electrophoresis, Polyacrylamide Gel; D051379:Mice; D008928:Mitochondria / Q000201:enzymology* / Q000235:genetics*; D008954:Models, Biological; D008969:Molecular Sequence Data; D009154:Mutation / Q000235:genetics; D009687:Nuclear Proteins / Q000378:metabolism; D021122:Protein Subunits / Q000235:genetics / Q000378:metabolism*; D013194:Staining and Labeling
D004272:DNA, Mitochondrial; D009687:Nuclear Proteins; D021122:Protein Subunits; D042967:Electron Transport Complex I
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