Key result
Tocainide suppressed after-contractions and EMG signals and partially reversed the histochemical muscle phenotype in ADR mutant mice, suggesting ADR is a myotonia.
Population
ADR (arrested development of righting response) mutant mice
Design
Preclinical
Authors
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Does not support clinical use of tocainide in myotonia; leaves open whether ADR mice model human disease.
The ADR mouse mutant exhibits physiological and histochemical properties characteristic of myotonia, which can be partially reversed by the membrane-stabilizing drug tocainide.
Reininghaus et al. (1988) studied Hereditary neuromuscular syndrome (arrested development of righting response, ADR). Tocainide was evaluated on Muscle physiology and histochemistry (after-contractions, EMG signals, muscle phenotype). Tocainide suppressed after-contractions and EMG signals and partially reversed the histochemical muscle phenotype in ADR mutant mice, suggesting ADR is a myotonia.
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