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The exact mechanism or mechanisms by which acetazolamide ameliorates myotonia are unclear. These researchers studied the effects of acetazolamide on voltage dependency, chloride conductance, and intracellular acidification in cultured human embryo kidney cells transfected with the gene for the human chloride channel 1 (ClC-1).
The researchers superfused cells containing the ClC-1 gene for 2 minutes with solutions containing either acetazolamide, phenytoin, or mexiletine. After acetazolamide superfusion, measurements of instantaneous and steady-state currents demonstrated a shift in voltage dependency toward negative potentials, with a resulting increase in chloride conductance near the resting membrane potential. A 100-μM solution of acetazo…