Abstract
Currently, there is a paucity of available treatment strategies for oxidative phosphorylation disorders. Coenzyme Q10 (CoQ10) and related synthetic quinones are the only agents to date that have pr...
Highlights
The mitochondrial respiratory chain (MRC; Figure 1) is located in the inner mitochondrial membrane and consists of 4 enzyme complexes: complex I (NADH: ubiquinone reductase; EC 1.6.5.3), complex II (succinate: ubiquinone reductase; EC 1.3.5.1), complex III (ubiquinol: cytochrome c reductase; EC 1.10.2.2), and complex IV (cytochrome c oxidase; EC 1.9.3.1;).[1,2] The MRC together with complex V (adenosine triphosphate [ATP] synthase; EC 3.6.3.14) synthesizes ATP, the energy currency of the cell by the process of oxidative phosphorylation.[1,2] In addition to existing as discrete entities, recent studies have indicated that the MRC enzymes can exist as supercomplexes within the inner mitochondrial membrane consisting of aggregates of complexes I, III, and IV; complexes I and III; and complexes III and IV.[3]The MRC disorders are a heterogeneous group of multisystemic diseases that develop as the result of mutations in either nuclear or mitochondrial DNA.[2]
In addition to existing as discrete entities, recent studies have indicated that the mitochondrial respiratory chain (MRC) enzymes can exist as supercomplexes within the inner mitochondrial membrane consisting of aggregates of complexes I, III, and IV; complexes I and III; and complexes III and IV.[3]
The MRC disorders are a heterogeneous group of multisystemic diseases that develop as the result of mutations in either nuclear or mitochondrial DNA.[2]
Summary
The mitochondrial respiratory chain (MRC; Figure 1) is located in the inner mitochondrial membrane and consists of 4 enzyme complexes: complex I (NADH: ubiquinone reductase; EC 1.6.5.3), complex II (succinate: ubiquinone reductase; EC 1.3.5.1), complex III (ubiquinol: cytochrome c reductase; EC 1.10.2.2), and complex IV (cytochrome c oxidase; EC 1.9.3.1;).[1,2] The MRC together with complex V (adenosine triphosphate [ATP] synthase; EC 3.6.3.14) synthesizes ATP, the energy currency of the cell by the process of oxidative phosphorylation.[1,2] In addition to existing as discrete entities, recent studies have indicated that the MRC enzymes can exist as supercomplexes within the inner mitochondrial membrane consisting of aggregates of complexes I, III, and IV; complexes I and III; and complexes III and IV.[3]The MRC disorders are a heterogeneous group of multisystemic diseases that develop as the result of mutations in either nuclear or mitochondrial DNA.[2].
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