References
Query Results -- Details
MGI Accession ID: MGI:3699478
J Number: J:118365
Other Accession IDs:
Title: Reversal of neurological defects in a mouse model of Rett syndrome.
Authors: Guy J; Gan J; Selfridge J; Cobb S; Bird A
Journal: Science
Volume: 315
Issue: 5815
Date: 2007 Feb 23
Year: 2007
Pages: 1143-7
Review Status: Peer Reviewed
Abstract:
Rett syndrome is an autism spectrum disorder caused by mosaic expression of mutant copies of the X-linked MECP2 gene in neurons. However, neurons do not die, which suggests that this is not a neurodegenerative disorder. An important question for future therapeutic approaches to this and related disorders concerns phenotypic reversibility. Can viable but defective neurons be repaired, or is the damage done during development without normal MeCP2 irrevocable? Using a mouse model, we demonstrate robust phenotypic reversal, as activation of MeCP2 expression leads to striking loss of advanced neurological symptoms in both immature and mature adult animals.
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