A Patient Suffering from Hypokalemic Periodic Paralysis Is Deficient in Skeletal Muscle ATP-sensitive K+ channels

Sofija Jovanovic, Qingyou Du, Somnath Mukhopadhyay, Robert Swingler, Richard Buckley, Jane McEachen, Aleksandar Jovanovic

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    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.

    Original languageEnglish
    Pages (from-to)71-74
    Number of pages4
    JournalClinical and Translational Science
    Volume1
    Issue number1
    DOIs
    Publication statusPublished - May 2008

    Keywords

    • hypokalemic periodic paralysis
    • K-ATP channels
    • skeletal muscle
    • Kir6.2
    • POTASSIUM CHANNELS
    • H9C2 CELLS
    • EXPRESSION
    • ISCHEMIA
    • SUBUNIT
    • SERVES
    • KINASE

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