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  • Title: Identification and functional analysis of mitochondrial complex I assembly factor homologues in C. elegans.
    Author: van den Ecker D, van den Brand MA, Ariaans G, Hoffmann M, Bossinger O, Mayatepek E, Nijtmans LG, Distelmaier F.
    Journal: Mitochondrion; 2012 May; 12(3):399-405. PubMed ID: 22387847.
    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.
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