Huntington's Disease Lab Report

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Huntington disease skeletal muscle is hyperexcitable owing to chloride and potassium channel dysfunction For this experiment they wanted to exam how Huntington diseased muscle affects the contraction control in the membrane. They first measured the action potentials in the diseased mice and compared that to the wild type mice. In the R6/2 mice they noticed a significant lengthening (almost double) in the repolarization phase that was not present in the wild type mice. The fibers of the diseased mice were also more sensitive to current stimulation that was needed to produce an action potential versus the control mice. A spontaneous action potential occurred in the diseased mice as well after the depolarization. They hypothesized that the repolarization, hyperexcitability, and spontaneous action …show more content…

The results for their test using a three-pulse voltage clamp protocol showed significant chloride reductions in the R6/2 mice, compared to the control. This result confirmed their first hypothesis. A second hypothesis was that if self-triggered action potentials occur after a subthreshold stimuli (as was observed) then there must be hyperexcitability present in the Huntington’s disease muscle caused by a reduction in the inward flow of potassium conductance. To test this hypothesis they measured Kir currents from both the diseased and the control mice. Their results proved the second hypothesis correct because the R6/2 mice had significantly lower potassium conductance than the control mice. By focusing on the membrane itself within the muscle, this study was able to open up possible discoveries and treatments on how to control the horrible symptoms that go along with Huntington’s disease. Many of our treatments today in many different diseases start at the membrane and this may be able to

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