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Myocytes from congenital myotonic dystrophy display abnormal Na + channel activities
Author(s) -
Bernareggi Annalisa,
Furling Denis,
Mouly Vincent,
Ruzzier Fabio,
Sciancalepore Marina
Publication year - 2005
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.20235
Subject(s) - myotonic dystrophy , myocyte , myotonia , patch clamp , sodium channel , medicine , chemistry , endocrinology , biophysics , biology , electrophysiology , sodium , organic chemistry
Abstract 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. Muscle Nerve, 2005