Myotonic dystrophy CTG repeat expansion alters Ca2+ channel functional expression in PC12 cells.
FEBS Lett
; 581(23): 4430-8, 2007 Sep 18.
Article
em En
| MEDLINE
| ID: mdl-17719582
ABSTRACT
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.
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Base de dados:
MEDLINE
Assunto principal:
Canais de Cálcio
/
Proteínas Serina-Treonina Quinases
/
Expansão das Repetições de Trinucleotídeos
Limite:
Animals
Idioma:
En
Ano de publicação:
2007
Tipo de documento:
Article