Identification and functional analysis of mitochondrial complex I assembly factor homologues in C. elegans

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 demons...

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Bibliographic Details
Published in:Mitochondrion 2012-05, Vol.12 (3), p.399-405
Main Authors: van den Ecker, Daniela, van den Brand, Mariël A, Ariaans, Gerke, Hoffmann, Michael, Bossinger, Olaf, Mayatepek, Ertan, Nijtmans, Leo G, Distelmaier, Felix
Format: Article
Language:English
Subjects:
Animals
Caenorhabditis elegans - genetics
Caenorhabditis elegans - metabolism
Caenorhabditis elegans Proteins - genetics
Caenorhabditis elegans Proteins - metabolism
Electron Transport Complex I - genetics
Electron Transport Complex I - metabolism
Humans
Mitochondria - metabolism
Mitochondrial Proteins - genetics
Mitochondrial Proteins - metabolism
NADH Dehydrogenase - genetics
NADH Dehydrogenase - metabolism
Organelle Biogenesis
Sequence Homology, Amino Acid
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Summary: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.
ISSN:1567-7249
1872-8278
DOI:10.1016/j.mito.2012.01.003