[HTML][HTML] Proteomic and metabolomic analyses of mitochondrial complex I-deficient mouse model generated by spontaneous B2 short interspersed nuclear element …

DW Leong, JC Komen, CA Hewitt, E Arnaud… - Journal of Biological …, 2012 - ASBMB
Eukaryotic cells generate energy in the form of ATP, through a network of mitochondrial
complexes and electron carriers known as the oxidative phosphorylation system. In
mammals, mitochondrial complex I (CI) is the largest component of this system, comprising
45 different subunits encoded by mitochondrial and nuclear DNA. Humans diagnosed with
mutations in the gene NDUFS4, encoding a nuclear DNA-encoded subunit of CI (NADH
dehydrogenase ubiquinone Fe-S protein 4), typically suffer from Leigh syndrome, a …