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U.S.A."],"published-print":{"date-parts":[[2019,10,15]]},"abstract":"<jats:title>Significance<\/jats:title>\n                  <jats:p>We report on a single mutation in the \u03b11-subunit M2 helix (p.\u03b11Leu251Arg) of the muscle acetylcholine receptor (AChR) found in a patient with congenital myasthenic syndrome (CMS) that is shown to convert the AChR into chloride conductance at positive potentials. Constriction of the channel pore with partial desolvation and stabilization of the permeating chloride ions by the arginine residues is revealed as the underlying mechanism. This article is of general interest because it describes a mechanism for the transformation of the muscle AChR into an inhibitory channel, and presents a report of charge selectivity conversion in association with a naturally occurring single mutation. 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