Duchenne dystrophy viewed as a disturbance of nerve-muscle interactions.
The possibility that some features of Duchenne muscular dystrophy (DMD) can be explained as a disturbance of nerve-muscle interaction during development is discussed. It is argued that the initial disturbance in DMD is due to a slower rate of maturation of skeletal muscle fibers. Normally, the maturation of the motor reflexes is closely followed by maturation of the muscle fibers. The possibility is considered that if muscle fibers mature more slowly than normal, they are not able to withstand the type of activity imposed upon them by the mature motoneuron. This applies mainly to the high frequency activity imposed on the fibers of motor units that are destined to become "fast." Experimental evidence suggesting such a mechanism is presented.
D000818:Animals; D002118:Calcium / Q000378:metabolism; D002462:Cell Membrane / Q000502:physiology; D002648:Child; D006801:Humans; D007527:Isoenzymes; D007770:L-Lactate Dehydrogenase / Q000378:metabolism; D009046:Motor Neurons / Q000502:physiology; D024510:Muscle Development; D009132:Muscles / Q000378:metabolism / Q000473:pathology; D009136:Muscular Dystrophies / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D009137:Muscular Dystrophy, Animal; D009469:Neuromuscular Junction / Q000502:physiology*; D012018:Reflex / Q000502:physiology; D012519:Sarcoplasmic Reticulum / Q000378:metabolism; D013577:Syndrome
D007527:Isoenzymes; D007770:L-Lactate Dehydrogenase; D002118:Calcium