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Mitochondrial Disorders

Enzymatic analysis of the individual components of the mitochondrial electron transport chain and ubiquinone determination is routinely performed on small muscle biopsies (50-100mg). We ask that you contact the lab for sample preparation/transport details. In addition, we can assay very long chain acyl CoA dehydrogenase, carnitine palmitoyl transferase I and II and pyruvate dehydrogenase activities on small muscle biopsies. Small liver biopsies can also be processed.
Diagram of Mitochondrial Electron Transport Chain