Modest reactivation of the mutant FMR1 gene by valproic acid is accompanied by histone modifications but not DNA demethylation
E Tabolacci, I De Pascalis, M Accadia… - Pharmacogenetics …, 2008 - journals.lww.com
Fragile X syndrome (FXS), the leading cause of inherited mental retardation, is due to
expansion and methylation of a CGG sequence in the FMR1 gene, which result in its
silencing. We previously demonstrated a reactivation of FMR1 in FXS cells treated with the
DNA demethylating drug 5-azadeoxycytidine, and, to a lesser extent, with the histone
deacetylating drug butyrate. To identify other reactivating drugs, we now treated three FXS
lymphoblastoid cell lines with valproic acid (VPA), a well-known antiepileptic drug, causing …
expansion and methylation of a CGG sequence in the FMR1 gene, which result in its
silencing. We previously demonstrated a reactivation of FMR1 in FXS cells treated with the
DNA demethylating drug 5-azadeoxycytidine, and, to a lesser extent, with the histone
deacetylating drug butyrate. To identify other reactivating drugs, we now treated three FXS
lymphoblastoid cell lines with valproic acid (VPA), a well-known antiepileptic drug, causing …