A mouse Mecp2-null mutation causes neurological symptoms that mimic Rett syndrome
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منابع مشابه
Mecp2-Null Mice Provide New Neuronal Targets for Rett Syndrome
BACKGROUND Rett syndrome (RTT) is a complex neurological disorder that is one of the most frequent causes of mental retardation in women. A great landmark in research in this field was the discovery of a relationship between the disease and the presence of mutations in the gene that codes for the methyl-CpG binding protein 2 (MeCP2). Currently, MeCP2 is thought to act as a transcriptional repre...
متن کاملRett syndrome: a surprising result of mutation in MECP2.
The identification of mutations in the gene encoding methyl CpG binding protein 2 (MeCP2) in Rett syndrome represents a major advance in the field. The current model predicts that MeCP2 represses transcription by binding methylated CpG residues and mediating chromatin remodeling. A physical interaction between MeCP2, histone deacetylases and the transcriptional co-repressor Sin3A has been demon...
متن کاملSevere changes in colon epithelium in the Mecp2-null mouse model of Rett syndrome
BACKGROUND Rett syndrome is best known due to its severe and devastating symptoms in the central nervous system. It is produced by mutations affecting the Mecp2 gene that codes for a transcription factor. Nevertheless, evidence for MECP2 activity has been reported for tissues other than those of the central nervous system. Patients affected by Rett presented with intestinal affections whose ori...
متن کاملHippocampal synaptic plasticity is impaired in the Mecp2-null mouse model of Rett syndrome.
Rett syndrome is an X-linked neurodevelopmental disorder caused by mutations in the gene encoding the transcriptional repressor methyl-CpG-binding protein 2 (MeCP2). Here we demonstrate that the Mecp2-null mouse model of Rett syndrome shows an age-dependent impairment in hippocampal CA1 long-term potentiation induced by tetanic or theta-burst stimulation. Long-term depression induced by repetit...
متن کاملCharacterization of the MeCP2 Knockin Mouse Model for Rett Syndrome
Rett syndrome, one of the most common causes of mental retardation in females, is caused by mutations in the X chromosomal gene MECP2. Mice deficient for MeCP2 recapitulate some of the symptoms seen in patients with Rett syndrome. It has been shown that reactivation of silent MECP2 alleles can reverse some of the symptoms in these mice. We have generated a knockin mouse model for translational ...
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ژورنال
عنوان ژورنال: Nature Genetics
سال: 2001
ISSN: 1061-4036,1546-1718
DOI: 10.1038/85899