title: Potassium currents in human myogenic cells from healthy and congenital myotonic dystrophy foetuses.
abstract: The whole-cell patch clamp technique was used to record potassium currents in in vitro differentiating myoblasts isolated from healthy and myotonic dystrophy type 1 (DM1) foetuses carrying 2000 CTG repeats. The fusion of the DM1 myoblasts was reduced in comparison to that of the control cells. The dystrophic muscle cells expressed less voltage-activated K(+) (delayed rectifier and non-inactivating delayed rectifier) and inward rectifier channels than the age-matched control cells. However, the resting membrane potential was not significantly different between the control and the DM1 cells. After four days in a differentiation medium, the dystrophic cells expressed the fast-inactivating transient outward K(+) channels, which were not observed in healthy cells. We suggest that the low level of potassium currents measured in differentiated DM1 cells could be related to their impaired fusion.
authors.affiliation: Department of Biophysics, Warsaw University of Life Sciences SGGW, Warsaw, Poland, ewa_nurowska@sggw.pl
authors.forename: Ewa
authors.initials: E
authors.lastname: Nurowska
authors.forename: Andrew
authors.initials: A
authors.lastname: Constanti
authors.forename: Beata
authors.initials: B
authors.lastname: Dworakowska
authors.forename: Vincent
authors.initials: V
authors.lastname: Mouly
authors.forename: Denis
authors.initials: D
authors.lastname: Furling
authors.forename: Paola
authors.initials: P
authors.lastname: Lorenzon
authors.forename: Tiziana
authors.initials: T
authors.lastname: Pietrangelo
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Dołowy
authors.forename: Fabio
authors.initials: F
authors.lastname: Ruzzier
mesh_terms: D002462:Cell Membrane / Q000502:physiology; D055724:Electrophysiological Phenomena; D005333:Fetus / Q000166:cytology*; D006801:Humans; D009223:Myotonic Dystrophy / Q000473:pathology / Q000503:physiopathology*; D018408:Patch-Clamp Techniques; D024642:Potassium Channels, Voltage-Gated / Q000502:physiology*; D032496:Satellite Cells, Skeletal Muscle / Q000473:pathology / Q000502:physiology*
chemical_list: D024642:Potassium Channels, Voltage-Gated