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July 1961

Bioelectric Properties of Dystrophic Mammalian MuscleExcitability, Membrane Resting, and Action Potentials in Mouse Hereditary Myopathy

Arch Neurol. 1961;5(1):46-59. doi:10.1001/archneur.1961.00450130048007

Introduction  This study was designed to investigate the bioelectric properties of skeletal muscle afflicted with hereditary myopathy, dystrophia muscularis of the mouse, in an attempt to delineate the effects of this genetically determined disorder on the physiology of the involved muscles. The strain dy dy mice (derived from strain 129 of the Jackson Memorial Laboratories) was described first by Michelson, Russell, and Harman.18 The autosomal recessive inheritance develops in these animals as a progressive, severe wasting and weakness of extremity and trunk striated muscles, especially of the hind limbs, with spasmodic flexion and flaccid extension. Kyphosis becomes severe and death usually is premature, coming when the animal is between 1 and 8 months old. Because of the genetic origin, the absence of nervous system lesions, and the presence of characteristic histologic changes in the muscles, the disease is regarded as a primary myopathy similar to that of human progressive

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