Mice with truncated MeCP2 recapitulate many Rett syndrome features and display hyperacetylation of histone H3.

By M. Shahbazian, Juan I. Young, L. Yuva-Paylor, Corinne M. Spencer, B. Antalffy, J. Noebels, D. Armstrong, R. Paylor, H. Zoghbi. Neuron, 2002

794 citations recorded by the source corpus.

Subfields: Cognitive Neuroscience, Genetics, Molecular Biology

View Paper: https://www.semanticscholar.org/paper/b3bcfbfed963b093685b096a413cbf6a075586fd

How researchers see this paper

The researchers whose trusted networks rank it highest, strongest advocate first. Every researcher gets a different order.

In Cognitive Neuroscience

1. H. Zoghbi
2. Fred H. Gage
3. James Bradley Aimone
4. Maria H. Chahrour
5. Mark F. Bear
6. Michael E. Greenberg
7. Chunmei Zhao
8. A. Bird

In Genetics

1. H. Zoghbi
2. Maria H. Chahrour
3. Mark F. Bear
4. James Bradley Aimone
5. A. Bird
6. Michael E. Greenberg
7. Fred H. Gage
8. R. Jaenisch

In Molecular Biology

1. H. Zoghbi
2. A. Bird
3. Maria H. Chahrour
4. Michael E. Greenberg
5. Mark F. Bear
6. R. Jaenisch
7. Robert S. Illingworth
8. Xinyu Zhao

Citations in the corpus

• The story of Rett syndrome: from clinic to neurobiology. (2007, positive): “The RTT mouse model, Mecp2 308/Y , carrying a truncated Mecp2 allele, displays increased anxiety-like behavior (McGill et al., 2006; Shahbazian et al., 2002a).”
• Synaptic dysfunction in neurodevelopmental disorders associated with autism and intellectual disabilities. (2012, background): “Mice lacking functional MeCP2 reproduce features of RTT (Chen et al. 2001; Guy et al. 2001; Shahbazian et al. 2002a; Pelka et al. 2006).”
• Loss of silent-chromatin looping and impaired imprinting of DLX5 in Rett syndrome (2005, background): “Furthermore, mice that carry clinically relevant mutations in the C terminus of Mecp2 develop progressive neurological phenotypes similar to those of RTT but milder than those of Mecp2 -null mic”
• Mild overexpression of MeCP2 causes a progressive neurological disorder in mice. (2004, background): “The transgenic mice show enhanced coordination and cerebellar motor learning, whereas the MeCP2(308) mice suffer impaired coordination and cerebellar learning (25).”
• Brain-specific phosphorylation of MeCP2 regulates activity-dependent Bdnf transcription, dendritic growth, and spine maturation. (2006, background): “Knockout mouse models with disrupted MeCP2 function mimic many key clinical features of RTT, including normal early postnatal life followed by developmental regression that results in motor impairment, irregular breathing, hindlimb clasping, and early mortality (Chen et al., 2001; Guy et al., 2001; ”
• Epigenetic regulation of gene expression: how the genome integrates intrinsic and environmental signals (2003, background): “Mice with null mutations in Mecp2 show phenotypic similarities to the human condition, including delayed onset of symptoms, small brain size, reduced dendritic arborization, tremors, inertia, abnormal gait and arrhythmic breathin”
• Mecp2 deficiency leads to delayed maturation and altered gene expression in hippocampal neurons. (2007, positive): “Several lines of Mecp2 mutant mice (KO) have been generated and these mice develop similar symptoms to those seen in RTT patients and have been widely used to study the etiology of human RTT (Chen et al., 2001; Guy et al., 2001; Shahbazian et al., 2002; Pelka et al., 2006).”
• Neuronal MeCP2 is expressed at near histone-octamer levels and globally alters the chromatin state (2010, background): “It was previously reported that in the Mecp2 mutant mouse brain, levels of histone H3Ac are elevated (Shahbazian et al., 2002).”
• REST and its corepressors mediate plasticity of neuronal gene chromatin throughout neurogenesis. (2005, background): “Although MeCP2 represses genes outside and within the nervous system, inexplicably, the effects of MeCP2 mutations in RTT patients and mouse models are observed primarily in the nervous system (Chen et al., 2001; Guy et al., 2001; Shahbazian et al., 2002 ).”