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Updated: Jul 14, 2026

Levator Auris Longus Preparation for Examination of Mammalian Neuromuscular Transmission Under Voltage Clamp Conditions
Published on: May 5, 2018
Myotonia and neuromuscular transmission in the mouse
D Költgen1, H Brinkmeier, H Jockusch
1Developmental Biology Unit, University of Bielefeld, Germany.
Abstract:
The role of neuromuscular transmission and acetylcholine receptors in the phenotypic expression of hereditary myotonia was reinvestigated in two mutants of the mouse, ADR (adr/adr) and MTO (adrmto/adrmto). Three neuromuscular blockers, curare, flaxedil, and alpha-bungarotoxin, did not prevent mechanical myotonia of EDL and soleus muscles from the two mutants. Furthermore, electrical myotonia was demonstrated in isolated ADR muscle fibers devoid of nerve endings. We conclude that neither release nor reception of acetylcholine are important for the mechanism of myotonia in mouse mutants. The previously described suppression of myotonic aftercontractions by high concentrations of curare (Muscle & Nerve 1987;10:293-298) could not be reproduced; rather, a prolongation of aftercontractions was found. The other drugs had no significant effect on myotonic aftercontractions. Because neuromuscular transmission is not involved in human myotonias, this result supports the use of myotonic mice as a model, at least for recessive generalized myotonia (Becker).
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