Loss of MECP2 Leads to Activation of P53 and Neuronal Senescence.
Ontology highlight
ABSTRACT:
SUBMITTER: Ohashi M
PROVIDER: S-EPMC5995366 | biostudies-literature | 2018 May
REPOSITORIES: biostudies-literature
Publications
Loss of MECP2 Leads to Activation of P53 and Neuronal Senescence.
Ohashi Minori M Korsakova Elena E Allen Denise D Lee Peiyee P Fu Kai K Vargas Benni S BS Cinkornpumin Jessica J Salas Carlos C Park Jenny C JC Germanguz Igal I Langerman Justin J Chronis Contantinos C Kuoy Edward E Tran Stephen S Xiao Xinshu X Pellegrini Matteo M Plath Kathrin K Lowry William E WE
Stem cell reports 20180501 5
To determine the role for mutations of MECP2 in Rett syndrome, we generated isogenic lines of human induced pluripotent stem cells, neural progenitor cells, and neurons from patient fibroblasts with and without MECP2 expression in an attempt to recapitulate disease phenotypes in vitro. Molecular profiling uncovered neuronal-specific gene expression changes, including induction of a senescence-associated secretory phenotype (SASP) program. Patient-derived neurons made without MECP2 showed signs o ...[more]
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