Huntington disease skeletal muscle is hyperexcitable owing to chloride and potassium channel dysfunction.

  title={Huntington disease skeletal muscle is hyperexcitable owing to chloride and potassium channel dysfunction.},
  author={Christopher W Waters and Grigor Varuzhanyan and Robert J Talmadge and Andrew A Voss},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  volume={110 22},
Huntington disease is a progressive and fatal genetic disorder with debilitating motor and cognitive defects. Chorea, rigidity, dystonia, and muscle weakness are characteristic motor defects of the disease that are commonly attributed to central neurodegeneration. However, no previous study has examined the membrane properties that control contraction in Huntington disease muscle. We show primary defects in ex vivo adult skeletal muscle from the R6/2 transgenic mouse model of Huntington disease… CONTINUE READING
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