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

Cellular & Molecular Biology Letters

Denis Furling²  Tiziana Pietrangelo⁴  Andrew Constanti¹  Ewa Nurowska³  Fabio Ruzzier⁵  Beata Dworakowska³  Paola Lorenzon⁵  Krzysztof Dołowy³  Vincent Mouly² 

[1] Department of Pharmacology, The School of Pharmacy, London, Great Britain$$;UMR S787, Inserm / UPMC-Paris 6/Institute of Myology, Paris Cedex, France$$;Department of Biophysics, Warsaw University of Life Sciences SGGW, Warsaw, Poland$$;Department of Physiology and Pathology, University of Trieste, Trieste, Italy$$Interuniversity Institute of Myology (IIM), Center for Research on Ageing, University “G. d’Annunzio”, Chieti, Italy$$;Department of Physiology and Pathology, University of Trieste, Trieste, Italy$$

关键词: Potassium channels;    Myoblast fusion;    Congenital myotonic dystrophy;    Patch-clamp;   

DOI  :  10.2478/s11658-009-0006-4

学科分类：分子生物学，细胞生物学和基因

来源: Uniwersytet Wroclawski * Wydzial Biotechnologii / University of Wroclaw, Faculty of Biotechnology

【 摘 要 】

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.

【 授权许可】

Unknown