Referenced in: none

Automatically associated channels: Kir6.2

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

Authors: Sofija Jovanović, Qingyou Du, Somnath Mukhopadhyay, Robert Swingler, Richard Buckley, Jane McEachen, Aleksandar Jovanović

Journal, date & volume: Clin Transl Sci, 2008 May , 1, 71-4

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/20396605

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.