�ber den Muskel-Energie-Stoffwechsel bei Kindern mit progressiver Muskeldystrophie Typ Duchenne

Purine Metabolism in Fibroblasts of Patients with Duchenne’s Muscular Dystrophy

“…Quite contrary to the reported studies of intercellular metabolism of '• 'C-adenine in erythrocytes [Solomons et al, 1977] and muscles [Stengel-Rutkowski and Barthelmai, 1973;Thomson and Smith, 1978] of patients with DMD. we could not find any ATP-ADP deficiency in the metabolically converted ' 'C-adenine or "C-AMP in cul tured fibroblasts of these patients.…”

Increased Turnover of Muscle Contractile Proteins in Duchenne Muscular Dystrophy as Assessed by 3-Methylhistidine and Creatinine Excretion

“…Indeed the amount of muscle present could actually be less than calculated, because the increased creatinine excretion in dystrophic patients will result in an overestimation of muscle mass if any creatine is converted into creatinine by nonenzymic reactions in the urine (Benedict, Kalinsky, Scarrone, Wertheim & Stetten, 1955). Against this trend to overestimate muscle mass is the finding that concentrations of creatine phosphate, the normal source of creatinine, are lower than normal in biopsy samples of muscle from patients with Duchenne muscular dystrophy (Stengl-Rutkowski & Barthelmai, 1973). A lowered concentration of creatine phosphate probably results from the reduced actomyosin content of dystrophic muscle (Schapira, Dreyfus, Schapira & Kruh, 1955;Simon, Gross & Lessell, 1962), and indeed it is likely that creatinine excretion is a more accurate index of the mass of contractile elements (which also contains the 3-methylhistidine residues) than of total muscle.…”

Clinical Applications of Nuclear Magnetic Resonance Spectroscopy: A Review

“…For years investigators using conventional biochemical techniques (Vignos & Warner 1963, Stengel-Rutkowski & Barthelmai 1973, Thompson & Smith 1978 and even in vitro NMR methods (Glonek et al 1981) have considered that the concentrations of phosphocreatine and ATP in muscle are diminished in dystrophy. This information is important since if true, the possibility that the level of ATP may be increased by therapy (Thompson & Smith 1978) requires investigation.…”