Myocytes from congenital myotonic dystrophy display abnormal Na+ channel activities.
Na(+) currents were measured in myocytes from a fetus with congenital myotonic dystrophy type 1 (DM1) using the patch-clamp whole-cell technique. Steady-state activation and inactivation properties of Na(+) channels were not substantially different between these cells and age-matched control cells. However, a decrease in Na(+) channel density and a faster rate of recovery from inactivation were found in myocytes from congenital DM1 suggesting that changes in functional Na(+) channels may affect cell excitability of muscle cells of patients with this disorder.
Bernareggi
Annalisa
A
Department of Physiology and Pathology, University of Trieste, Via Fleming 22, 34127 Trieste, Italy.
Furling
Denis
D
Mouly
Vincent
V
Ruzzier
Fabio
F
Sciancalepore
Marina
M
D002478:Cells, Cultured; D005333:Fetus; D006801:Humans; D015640:Ion Channel Gating / Q000502:physiology; D008564:Membrane Potentials / Q000502:physiology; D018485:Muscle Fibers, Skeletal / Q000378:metabolism* / Q000473:pathology; D009223:Myotonic Dystrophy / Q000378:metabolism* / Q000503:physiopathology*; D015222:Sodium Channels / Q000502:physiology*
D015222:Sodium Channels