Possible role of apamin-sensitive K+ channels in myotonic dystrophy
| Autor: | Patricio Jalil, Alejandro Serani, Fernando Vergara, Maria I. Behrens, Osvaldo Alvarez |
|---|---|
| Rok vydání: | 1994 |
| Předmět: |
musculoskeletal diseases
Adult Male congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Potassium Channels Physiology Apamin complex mixtures Myotonic dystrophy Cellular and Molecular Neuroscience chemistry.chemical_compound Physiology (medical) Internal medicine medicine Humans Myotonic Dystrophy Myotonia congenita business.industry Electromyography Electric Conductivity Middle Aged Myotonia medicine.disease Muscle atrophy Potassium channel body regions Electrophysiology Endocrinology chemistry Chloride channel Female Neurology (clinical) medicine.symptom business Neuroscience |
| Zdroj: | Musclenerve. 17(11) |
| ISSN: | 0148-639X |
| Popis: | Myotonic muscular dystrophy is a genetic disease characterized mainly by muscle atrophy and myotonia, a repetitive electrical activity of muscle. In the present study, the possible role of apamin-sensitive K+ channels in the genesis of myotonia was investigated. Apamin is a peptide from bee venom that specifically blocks small conductance Ca(2+)-activated K+ channels. The injection of a small amount of apamin (20-30 microliters, 10 mumol/L) into the thenar muscle of myotonic dystrophy patients decreased the basal electrical activity during the electromyogram in the 6 patients studied. Myotonic discharges after muscle percussion were more difficult to trigger and of smaller intensity and duration. In 2 controls and in 2 patients with generalized myotonia, as well as in 1 patient with myotonia congenita (where the defect is in chloride channels), apamin had no effect. These results suggest that apamin-sensitive K+ channels participate in the mechanism that generates myotonia in myotonic dystrophy. |
| Databáze: | OpenAIRE |
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