西亚试剂：Subcomplex Iλ Specifically Controls Integrated Mitochondria

Subcomplex Iλ Specifically Controls Integrated Mitochondrial Functions in Caenorhabditis elegans

Marni J. Falk1*, Julie R. Rosenjack2, Erzsebet Polyak1, Wichit Suthammarak2, Zhongxue Chen3, Phil G. Morgan2,4, Margaret M. Sedensky2,4

1 Division of Human Genetics, Department of Pediatrics, The Children's Hospital of Philadelphia and University of Pennsylvania, Philadelphia, Pennsylvania, United States of America, 2 Department of Anesthesiology, Case Western Reserve University School of Medicine, Cleveland, Ohio, United States of America, 3 Biostatistics & Data Management Core, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America, 4 Department of Anesthesiology, Seattle Children's Regional Medical Center and University of Washington, Seattle, Washington, United States of America

Complex I dysfunction is a common, heterogeneous cause of human mitochondrial disease having poorly understood pathogenesis. The extensive conservation of complex I composition between humans and Caenorhabditis elegans permits analysis of individual subunit contribution to mitochondrial functions at both the whole animal and mitochondrial levels. We provide the first experimentally-verified compilation of complex I composition in C. elegans, demonstrating 84% conservation with human complex I. Individual subunit contribution to mitochondrial respiratory capacity, holocomplex I assembly, and animal anesthetic behavior was studied in C. elegans by RNA interference-generated knockdown of nuclear genes encoding 28 complex I structural subunits and 2 assembly factors. Not all complex I subunits directly impact respiratory capacity. Subcomplex Iλ subunits along the electron transfer pathway specifically control whole animal anesthetic sensitivity and complex II upregulation, proportionate to their relative impairment of complex I-dependent oxidative capacity. Translational analysis of complex I dysfunction facilitates mechanistic understanding of individual gene contribution to mitochondrial disease. We demonstrate that functional consequences of complex I deficiency vary with the particular subunit that is defective.

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