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. 2009 Feb 4;14(2):336–346. doi: 10.2478/s11658-009-0006-4

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

Ewa Nurowska 1,✉, Andrew Constanti 2, Beata Dworakowska 1, Vincent Mouly 3, Denis Furling 3, Paola Lorenzon 4, Tiziana Pietrangelo 4,5, Krzysztof Dołowy 1, Fabio Ruzzier 4
PMCID: PMC6275736  PMID: 19194665

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.

Key words: Potassium channels, Myoblast fusion, Congenital myotonic dystrophy, Patch-clamp

Full Text

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Abbreviation

DM1

myotonic dystrophy type 1

DMED

differentiation medium

DMPK

dystrophia myotonica protein kinase

IK(DR)

delayed rectifier current

IK(IR)

inward rectifier current

IK(TO)

transient outward K+ current

TEACl

tetraethylammonium chloride

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