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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.

Abbreviations

DM1:

myotonic dystrophy type 1

DMED:

differentiation medium

DMPK:

dystrophia myotonica protein kinase

I K(DR) :

delayed rectifier current

I K(IR) :

inward rectifier current

I K(TO) :

transient outward K+ current

TEACl:

tetraethylammonium chloride

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Correspondence to Ewa Nurowska.

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Nurowska, E., Constanti, A., Dworakowska, B. et al. Potassium currents in human myogenic cells from healthy and congenital myotonic dystrophy foetuses. Cell Mol Biol Lett 14, 336–346 (2009). https://doi.org/10.2478/s11658-009-0006-4

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  • DOI: https://doi.org/10.2478/s11658-009-0006-4

Key words

  • Potassium channels
  • Myoblast fusion
  • Congenital myotonic dystrophy
  • Patch-clamp