| Literature DB >> 22387847 |
Daniela van den Ecker1, Mariël A van den Brand, Gerke Ariaans, Michael Hoffmann, Olaf Bossinger, Ertan Mayatepek, Leo G Nijtmans, Felix Distelmaier.
Abstract
The biogenesis of mitochondrial NADH:ubiquinone oxidoreductase (complex I) requires several assembly chaperones. These so-called complex I assembly factors have emerged as a new class of human disease genes. Here, we identified putative assembly factor homologues in Caenorhabditis elegans. We demonstrate that two candidates (C50B8.3/NUAF-1, homologue of NDUFAF1 and R07H5.3/NUAF-3, homologue of NDUFAF3) clearly affect complex I function. Assembly factor deficient worms were shorter, showed a diminished brood size and displayed reduced fat content. Our results suggest that mitochondrial complex I biogenesis is evolutionarily conserved. Moreover, Caenorhabditis elegans appears to be a promising model organism to study assembly factor related human diseases.Entities:
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Year: 2012 PMID： 22387847 DOI： 10.1016/j.mito.2012.01.003
Source DB: PubMed Journal: Mitochondrion ISSN： 1567-7249 Impact factor: 4.160