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.
- hypokalemic periodic paralysis
- K-ATP channels
- skeletal muscle
- POTASSIUM CHANNELS
- H9C2 CELLS