Isotachophoretic Analysis of Some Compounds Involved in Energy Metabolism in Normal and Pathological Human Muscle Extracts
A number of muscular diseases seem to be associated with disturbances in purine metabolism. Altered purine metabolite compositions have been described for instance in Duchenne-type muscular dystrophy (DMD) (1) and malignant hyperthermia (2). For clarification of the possible metabolic defects as well as for diagnostic reasons it is important to know levels and variations in metabolites in muscle biopsies of control persons as well as patients suffering from muscular diseases. Isotachophoresis is a convenient technique for these analyses because, apart from nucleotides, other muscle metabolites important in energy metabolism such as orthophosphate (Pi), phosphocreatine (PCr) and lactate can be detected in a single run of about twenty minutes.
KeywordsControl Person Malignant Hyperthermia Energy Charge Caproic Acid Purine Metabolism
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