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Muscle-Type Phosphorylase Activity Present in Muscle Cells Cultured From Three Patients With Myophosphorylase Deficiency
Marie Claire Meienhofer, PhD;
Valerie Askanas, MD, PhD;
Dominique Proux-Daegelen, MS;
Jean-Claude Dreyfus, MD, PhD;
W. King Engel, MD
Arch Neurol. 1977;34(12):779-781.
Abstract
• Skeletal muscle fibers cultured from three patients whose mature fibers are deficient in glycogen myophosphorylase (EC 2.4.1.1) were shown to become rather mature, to have no excessive glycogen accumulation, and to develop significant myophosphorylase activity. That activity was characterized electrophoretically and immunologically and shown to be muscle phosphorylase rather than a genetically different type, thereby demonstrating true "rejuvenation" in culture of an enzyme genetically programmed ultimately to be deficient.
Author Affiliations
From the Institut de Pathologie Moleculaire (Drs Meienhofer, Proux-Daegelen, and Dreyfus), Paris, the Institute of Rehabilitation Medicine, New York University School of Medicine (Dr Askanas), New York, and the National Institutes of Health (Dr Engel), Bethesda, Md.
Footnotes
Accepted for publication May 3, 1977.
Reprint requests to 10-D-18, Clinical Center, National Institutes of Health, Bethesda, MD 20014 (Dr Engel).
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