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

• Autorzy: Nurowska E , Constanti A. , Dworakowska B. , Mouly V. , Furling D. , Lorenzon P. , Pietrangelo T. , Dolowy K. , Ruzzier F.
• Języki publikacji: EN

Abstrakty

EN

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.

Słowa kluczowe

EN

potassium channel; man; myogenic cell; myoblast fusion; congenital myotonic dystrophy; muscle cell; patch clamp

• Wydawca: -
• Czasopismo: Cellular and Molecular Biology Letters
• Rocznik: 2009
• Tom: 14
• Numer: 2
• Opis fizyczny: p.336-346,fig.,ref.

Twórcy

autor

Nurowska E

• Warsaw University of Life Sciences SGGW, Warsaw, Poland

autor

Constanti A.

autor

Dworakowska B.

autor

Mouly V.

autor

Furling D.

autor

Lorenzon P.

autor

Pietrangelo T.

autor

Dolowy K.

autor

Ruzzier F.

Bibliografia

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