Article
Histone Deacetylase Inhibitors Ameliorate Morphological Defects and Hypoexcitability of iPSC-Neurons from Rubinstein-Taybi Patients.
International journal of molecular sciences - 28 May 2021
Alari Valentina, Scalmani Paolo, Ajmone Paola Francesca, Perego Sara, Avignone Sabrina, Catusi Ilaria, Lonati Paola Adele, Borghi Maria Orietta, Finelli Palma, Terragni Benedetta, Mantegazza Massimo, Russo Silvia, Larizza Lidia
Abstract excerpt
Rubinstein-Taybi syndrome (RSTS) is a rare neurodevelopmental disorder caused by mutations in CREBBP or EP300 genes encoding CBP/p300 lysine acetyltransferases. We investigated the efficacy of the histone deacetylase inhibitor (HDACi) Trichostatin A (TSA) in ameliorating morphological abnormalities of iPSC-derived young neurons from P149 and P34 CREBBP-mutated patients and hypoexcitability of mature neurons from...
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