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.
|Uncontrolled Keywords:||Potassium channels - Myoblast fusion - Congenital myotonic dystrophy - Patch-clamp|
|Departments, units and centres:||Department of Pharmacology > Department of Pharmacology|
|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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