Biochemical analyses


Biochemical analyses of defects of mitochondrial energy metabolism involve two steps:


1. Screening of mitochondrial energy metabolism of intact mitochondria

Methods: Respirometry with permeabilized muscle fibres as well as radiochemical assay to determine substrate oxidation in native muscle homogenates
Specimen: at least 30 mg native, unfrozen muscle tissue
Shipment: Send specimen in a special MITO-shipping buffer provided by our laboratory at 0°C on water/ice mixture in a thermally isolated container (see also Shipping instructions)


2. Measurement of individual enzymes of mitochondrial energy metabolism

Specimen: at least 20 mg muscle tissue or fibroblast culture
Shipment: Send collected tissue on dry ice in a thermally isolated container (see also Shipping instructions)
If screening of mitochondrial energy metabolism is also requested, submission of additional tissue is not necessary.

Nomenclature of enzymes investigated in mitochondrial energy metabolism

Short term Enzyme-energy metabolism
PDHC pyruvate dehydrogenase complex
complex I NADH-ubiquinone oxidoreductase
complex I+III NADH-cytochrome c oxidoreductase
complex II succinate-ubiquinone oxidoreductase
complex II+III succinate-cytochrome c oxidoreductase
complex III ubiquinone-cytochrome c oxidoreductase
complex IV cytochrome c oxidase
complex V ATPase, oligomycin-sensitive
citrate synthase reference enzyme for energy metabolism



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