Potassium currents in human myogenic cells from healthy and congenital myotonic dystrophy foetuses

Nurowska, E., Constanti, A., Dworakowska, B., Mouly, V., Furling, D., Lorenzon, P., Pietrangelo, T., Dołowy, K. and Ruzzier, F. (2009) Potassium currents in human myogenic cells from healthy and congenital myotonic dystrophy foetuses. Cellular & Molecular Biology Letters, 14 (2). pp. 336-346.

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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.

Item Type:Article
Uncontrolled Keywords:Potassium channels - Myoblast fusion - Congenital myotonic dystrophy - Patch-clamp
Departments, units and centres:Department of Pharmacology > Department of Pharmacology
ID Code:3287
Journal or Publication Title:Cellular & Molecular Biology Letters
Deposited By:Library Staff
Deposited On:18 May 2012 16:55
Last Modified:18 May 2012 17:26

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