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

Authors :
Nurowska, Ewa
Constanti, Andrew
Dworakowska, Beata
Mouly, Vincent
Furling, Denis
Lorenzon, Paola
Pietrangelo, Tiziana
Dołowy, Krzysztof
Ruzzier, Fabio
Source :
Cellular & Molecular Biology Letters; Jun2009, Vol. 14 Issue 2, p336-346, 11p
Publication Year :
2009

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<superscript>+</superscript> (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<superscript>+</superscript> 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. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
14258153
Volume :
14
Issue :
2
Database :
Complementary Index
Journal :
Cellular & Molecular Biology Letters
Publication Type :
Academic Journal
Accession number :
49560683
Full Text :
https://doi.org/10.2478/s11658-009-0006-4