The dystrophin connection--ATP?
Clinical evidence is presented supporting the hypothesis that the metabolic abnormality in the dystrophin-defective muscular dystrophies (DMD and BMD) involves the ATP pathway. Objective laboratory data show corrective trends in the abnormal values of parameters relating to creatine and calcium metabolism (ATP) by use of glucagon-stimulated c-AMP and by use of synthetically produced adenylosuccinic acid (ASA). Disease accelerating mechanisms as suggested by analysis of the clinical features, and the therapeutic potential of ASA are discussed.
Bonsett
C A
CA
Department of Neurology, Indiana University School of Medicine, Indianapolis 46223.
Rudman
A
A
D000255:Adenosine Triphosphate / Q000378:metabolism*; D000367:Age Factors; D000375:Aging / Q000378:metabolism; D003402:Creatine Kinase / Q000097:blood; D000242:Cyclic AMP / Q000378:metabolism; D016189:Dystrophin / Q000172:deficiency / Q000235:genetics / Q000502:physiology*; D006801:Humans; D008954:Models, Biological; D009132:Muscles / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology; D009136:Muscular Dystrophies / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology*; D012016:Reference Values
D016189:Dystrophin; D000255:Adenosine Triphosphate; D000242:Cyclic AMP; D003402:Creatine Kinase