title: Ion transport systems in erythrocytes from paramyotonia patients.
abstract: To test the hypothesis of a general defect in the ion transport systems in the cell membranes of patients with paramyotonia congenita, we measured the ouabain-inhibitable and the furosemide-inhibitable K+ influxes in erythrocytes from two patients. In the temperature range examined (15-40 degrees C), the ouabain-inhibitable flux did not differ significantly from control. Also the furosemide-inhibitable flux was well in the rather large control range. The dependence of these fluxes on the external K+ concentration was normal. Energy depletion of the erythrocytes by preincubation with desoxy-glucose led to reduced K+ influxes that were not significantly different in patients and controls. The results do not support the hypothesis of a general defect in the ion transport systems in paramyotonia; they do, however, not rule out the possibility that the ion transport systems in paramyotonic skeletal muscle are defect.
mesh_terms: D000328:Adult; D001693:Biological Transport, Active / Q000187:drug effects; D004734:Energy Metabolism; D004912:Erythrocytes / Q000378:metabolism*; D005260:Female; D005665:Furosemide / Q000494:pharmacology; D006801:Humans; D066298:In Vitro Techniques; D007473:Ion Channels / Q000187:drug effects / Q000378:metabolism; D008297:Male; D009224:Myotonia Congenita / Q000097:blood*; D010042:Ouabain / Q000494:pharmacology; D011188:Potassium / Q000097:blood*; D012964:Sodium / Q000097:blood; D013696:Temperature
chemical_list: D007473:Ion Channels; D010042:Ouabain; D005665:Furosemide; D012964:Sodium; D011188:Potassium