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A patient suffering from hypokalemic periodic paralysis is deficient in skeletal muscle ATP-sensitive K channels.
- Source :
-
Clinical and translational science [Clin Transl Sci] 2008 May; Vol. 1 (1), pp. 71-4. - Publication Year :
- 2008
-
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.
- Subjects :
- Case-Control Studies
DNA Primers chemistry
Humans
Hypokalemia pathology
Male
Middle Aged
Muscle, Skeletal metabolism
Potassium chemistry
Potassium Channels, Inwardly Rectifying genetics
RNA, Messenger metabolism
Reverse Transcriptase Polymerase Chain Reaction
Hypokalemic Periodic Paralysis pathology
KATP Channels deficiency
KATP Channels metabolism
Muscle, Skeletal pathology
Potassium Channels, Inwardly Rectifying biosynthesis
Subjects
Details
- Language :
- English
- ISSN :
- 1752-8062
- Volume :
- 1
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Clinical and translational science
- Publication Type :
- Academic Journal
- Accession number :
- 20396605
- Full Text :
- https://doi.org/10.1111/j.1752-8062.2008.00007.x