Electrophoresis techniques to investigate defects in oxidative phosphorylation

Defects in mitochondrial oxidative phosphorylation (OXPHOS) are a frequent cause of severe inherited metabolic disorders and also contribute to aging. The OXPHOS system constitutes five multi-subunit complexes embedded in the mitochondrial inner membrane. Correct function of this system requires pro...

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Veröffentlicht in:	Methods (San Diego, Calif.) Calif.), 2008-12, Vol.46 (4), p.281-287
Hauptverfasser:	Calvaruso, Maria Antonietta, Smeitink, Jan, Nijtmans, Leo
Format:	Artikel
Sprache:	eng
Schlagworte:	Blotting, Western Blue native electrophoresis Electron Transport Electrophoresis - methods Electrophoresis, Gel, Two-Dimensional - methods Electrophoresis, Polyacrylamide Gel - methods Enzyme complexes Fibroblasts - ultrastructure Humans Mitochondria Mitochondria, Muscle - metabolism Mitochondrial diseases Mitochondrial Diseases - metabolism Oxidative Phosphorylation Two dimensional electrophorsis
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