Myotonic dystrophy CTG repeat expansion alters Ca2+channel functional expression in PC12 cells

Arturo Andrade, Mario Bermúdez de León, Oscar Hernández-Hernández, Bulmaro Cisneros, Ricardo Felix

Resultado de la investigaciónrevisión exhaustiva

9 Citas (Scopus)


We previously reported that expression of myotonic dystrophy (DM1) expanded CUG repeats impedes NGF-induced differentiation in a PC12 clone (CTG90 cells). Here, we present evidence for changes in the fractional contribution of distinct voltage-gated Ca 2+ channels, key elements in neurotrophin-promoted differentiation, to the total Ca 2+ current in the CTG90 cells. Patch-clamp recordings showed that the relative proportion of pharmacologically isolated Ca 2+ channel types differed between control and CTG90 cells. Particularly, the functional expression of N-type channels was significantly reduced. Though quantitative real-time RT-PCR revealed that transcripts for the pore-forming subunit encoding the N-type channels remained unchanged, the protein level analyzed by semi-quantitative Western blotting was down-regulated in the CTG90 cells. These data suggest modifications in the processing of N-type Ca 2+ channels in PC12 cells expressing the DM1 mutation.

Idioma originalEnglish
Páginas (desde-hasta)4430-4438
Número de páginas9
PublicaciónFEBS Letters
EstadoPublished - 18 sept. 2007
Publicado de forma externa

All Science Journal Classification (ASJC) codes

  • Biofísica
  • Biología estructural
  • Bioquímica
  • Biología molecular
  • Genética
  • Biología celular


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