Ontology highlight
ABSTRACT:
SUBMITTER: Cheval H
PROVIDER: S-EPMC3412380 | biostudies-literature | 2012 Sep
REPOSITORIES: biostudies-literature
Cheval Hélène H Guy Jacky J Merusi Cara C De Sousa Dina D Selfridge Jim J Bird Adrian A
Human molecular genetics 20120531 17
Rett Syndrome is a neurological disorder caused by mutations in the X-linked MECP2 gene. Mouse models where Mecp2 is inactivated or mutated recapitulate several features of the disorder and have demonstrated a requirement for the protein to ensure brain function in adult mice. We deleted the Mecp2 gene in ~80% of brain cells at three postnatal ages to determine whether the need for MeCP2 varies with age. Inactivation at all three time points induced Rett-like phenotypes and caused premature deat ...[more]