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The first important finding in the myotonic goats was that the unusual muscle stiffness was associated with long runs of action potentials in the muscle fibers. But what produced those action potentials?
2 maj 2020 · In 1970, the study of the pathomechanisms underlying myotonia in muscle fibers isolated from myotonic goats highlighted the importance of chloride conductance for skeletal muscle function; 20 years later, the human ClC-1 chloride channel has been cloned; last year, the crystal structure of human protein has been solved.
Muscle biopsy specimens from the myotonic goat, an animal model of heritable myotonia, were examined histochemically and by electron microscopy. After Periodic acid-Schiff (PAS) staining with diastase digestion, there was increased PAS-positive material within myotonic goat fibers, as compared with those of normal goats.
Myotonic potentials are defined as repetitive discharges, firing at a rate of 20–80 Hz, in which the amplitude and frequency of the potentials wax and wane, creating a characteristic “dive bomber” sound. 74 The individual potentials may resemble fibrillation potentials or positive sharp waves, but it is the repetitive firing and unique ...
genetic studies on murine (8, 9) and human (10-13) myotonia confirmed structural and functional defects in a skeletal muscle chloride channel (termed CIC-1). In view of the pivotal role played by the myotonic goat in establishing the pathophysiology of myotonia, we sought to define the molecular basis for diminished gcl in the myotonic
Myotonic does for sustainable meat goat doe perfor-mance under limited-input management conditions. Myotonic does maintained the lowest FEC among all doe breeds and warrant further evaluation as a genetic resource for controlling gastrointestinal parasitism. Key words: Boer, breed, health, Kiko, Myotonic, reproduction
The electrophysiology of mytonia, with a review of congenital myotonia of goats New Developments in Electromyography and Clinical Neurophysiology. S. Karger. Basel , Switzerland . Raven Press. New York , N.Y . Butterworths. London , England .
