Accessory subunit NUYM (NDUFS4) is required for stability of the electron input module and activity of mitochondrial complex I
作者： Flora Kahlhöfer , Katarzyna Kmita , Ilka Wittig , Klaus Zwicker , Volker Zickermann
DOI: 10.1016/J.BBABIO.2016.11.010
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摘要: Abstract Mitochondrial complex I is an intricate 1 MDa membrane protein with a central role in aerobic energy metabolism. The minimal form of consists fourteen subunits that are conserved from bacteria to man. In addition, eukaryotic comprises some 30 accessory largely unknown function. gene for the NDUFS4 subunit human hot spot fatal pathogenic mutations humans. We have deleted orthologous NUYM yeast Yarrowia lipolytica , established model system study and linked diseases. observed assembly which lacked only retained weak interaction factor N7BML (human NDUFAF2). Absence caused distortion iron sulfur clusters electron input domain leading decreased activity increased release reactive oxygen species. conclude has important stabilizing function module essential proper
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