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- W2108049416 endingPage "515" @default.
- W2108049416 startingPage "499" @default.
- W2108049416 abstract "Oxidative phosphorylation (OXPHOS) has a prominent role in energy metabolism of the cell. Being under bigenomic control, correct biogenesis and functioning of the OXPHOS system is dependent on the finely tuned interaction between the nuclear and the mitochondrial genome. This suggests that disturbances of the system can be caused by numerous genetic defects and can result in a variety of metabolic and biochemical alterations. Consequently, OXPHOS deficiencies manifest as a broad clinical spectrum. Complex I, the biggest and most complicated enzyme complex of the OXPHOS system, has been subjected to thorough investigation in recent years. Significant progress has been made in the field of structure, composition, assembly, and pathology. Important gains in the understanding of the Goliath of the OXPHOS system are: exposing the electron transfer mechanism and solving the crystal structure of the peripheral arm, characterization of almost all subunits and some of their functions, and creating models to elucidate the assembly process with concomitant identification of assembly chaperones. Unravelling the intricate mechanisms underlying the functioning of this membrane-bound enzyme complex in health and disease will pave the way for developing adequate diagnostic procedures and advanced therapeutic treatment strategies." @default.
- W2108049416 created "2016-06-24" @default.
- W2108049416 creator A5003287268 @default.
- W2108049416 creator A5014162862 @default.
- W2108049416 creator A5062064518 @default.
- W2108049416 creator A5085144984 @default.
- W2108049416 date "2006-07-11" @default.
- W2108049416 modified "2023-10-14" @default.
- W2108049416 title "Mitochondrial complex I: Structure, function and pathology" @default.
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