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A patient suffering from hypokalemic periodic paralysis is deficient in skeletal muscle ATP-sensitive K channels. | LitMetric

AI Article Synopsis

  • HOPP is a rare condition characterized by episodes of muscle weakness and low potassium levels (hypokalemia).
  • Researchers found that a patient with HOPP had a deficiency in certain potassium channels (K(ATP) channels) in their muscle membranes.
  • The study suggests that reduced levels of Kir6.2, a key component of these channels, could lead to the observed muscle weakness and symptoms in HOPP patients.

Article Abstract

Hypokalemic periodic paralysis (HOPP) is a rare disease associated with attacks of muscle weakness and hypokalemia. In the present study, immunoprecipitation/Western blotting has shown that a HOPP patient was deficient in sarcolemmal K(ATP) channels. Real-time RT-PCR has revealed that HOPP has decreased mRNA levels of Kir6.2, a pore-forming K(ATP) channel subunit, without affecting the expression of other K(ATP) channel-forming proteins. Based on these findings, we conclude that HOPP could be associated with impaired expression of Kir6.2 which leads to deficiency in skeletal muscle K(ATP) channels, which may explain the symptoms and clinical signs of this disease.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2854805PMC
http://dx.doi.org/10.1111/j.1752-8062.2008.00007.xDOI Listing

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