Accessory subunit NUYM (NDUFS4) is required for stability of the electron input module and activity of mitochondrial complex I

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• 作者：Flora Kahlhö ; fer^a ; ¹ ; Katarzyna Kmita^a ; ¹ ; Ilka Wittig^b ; ^d ; Klaus Zwicker^c ; Volker Zickermann^a ; ^d ; ^{Zickermann@med.uni-frankfurt.de}
• 关键词：Complex I assembly ; N7BML ; NDUFAF2 ; Iron sulfur cluster ; NADH ; ubiquinone oxidoreductase ; ROS
• 刊名：Biochimica et Biophysica Acta (BBA) - Bioenergetics
• 出版年：2017
• 出版时间：February 2017
• 年：2017
• 卷：1858
• 期：2
• 页码：175-181
• 全文大小：798 K
• 卷排序：1858

文摘

We deleted the gene for NUYM (NDUFS4) of mitochondrial complex I in Yarrowia lipolytica. We observed distortion of two iron-sulfur clusters and increased ROS formation. Assembly factor N7BML (NDUFAF2) remained bound to complex I. The data shed new light on pathogenic NDUFS4 mutations in humans.