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

详细信息	查看全文 | 推荐本文 |

• 作者：Daniela van den Ecker^a ; Marië ; l A. van den Brand^b ; Gerke Ariaans^a ; Michael Hoffmann^a ; Olaf Bossinger^c ; Ertan Mayatepek^a ; Leo G. Nijtmans^b ; Felix Distelmaier^a ; ^{felix.distelmaier@med.uni-duesseldorf.de}
• 关键词：BN-PAGE ; blue native polyacrylamide gel electrophoresis ; Complex I ; NADH ; ubiquinone oxidoreductase ; NDUF ; NADH dehydrogenase ubiquinone flavoprotein ; NGM ; Nematode Growth Medium ; NUAF-1/NUAF-3 ; NADH ; Ubiquinone oxidoreductase Assembly Factor 1 and 2 ; OXPHO
• 刊名：Mitochondrion
• 出版年：2012
• 期刊代码：39_15677249
• 类别：neu
• 出版时间：May, 2012
• 卷：12
• 期：3
• 页码：399-405
• 文件大小：620 K

摘要

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.