Mild overexpression of MeCP2 causes a progressive neurological disorder in mice.

By A. L. Collins, J. Levenson, Alexander P. Vilaythong, R. Richman, D. Armstrong, J. Noebels, J. David Sweatt, H. Zoghbi. Human Molecular Genetics, 2004

644 citations recorded by the source corpus.

Subfields: Cognitive Neuroscience, Genetics, Molecular Biology

View Paper: https://www.semanticscholar.org/paper/6f278ca661a317b3146941d05a52ce63e508e864

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. Mark F. Bear
3. Maria H. Chahrour
4. Michael E. Greenberg
5. J. Sweatt
6. Yangling Mu
7. A. Bird
8. Fred H. Gage

In Genetics

1. H. Zoghbi
2. Mark F. Bear
3. Michael E. Greenberg
4. Maria H. Chahrour
5. Robert S. Illingworth
6. J. Sweatt
7. A. Bird
8. Gene W. Yeo

In Molecular Biology

1. H. Zoghbi
2. Michael E. Greenberg
3. Fred H. Gage
4. A. Bird
5. Maria H. Chahrour
6. Mark F. Bear
7. Robert S. Illingworth
8. M. Marchetto

Citations in the corpus

• The story of Rett syndrome: from clinic to neurobiology. (2007, positive): “Studies of these mouse models suggest that MeCP2 levels must be tightly regulated even postnatally and that the slightest perturbation results in deleterious neurological consequences (Collins et al., 2004; Luikenhuis et al., 2004).”
• Synaptic dysfunction in neurodevelopmental disorders associated with autism and intellectual disabilities. (2012, background): “One particularly interesting phenotype reported in the Tsc2 +/ − mouse is an enhancement of late-phase LTP (Ehninger et al. 2008a).”
• Duplication of the MECP2 region is a frequent cause of severe mental retardation and progressive neurological symptoms in males. (2005, background): “Recently, a transgenic mouse model that overexpresses the human MECP2 gene under the control of its own promoter was reported (Collins et al. 2004).”
• Covalent modification of DNA regulates memory formation. (2007, neutral): “Rett syndrome is associated with mutations in MeCP2, one of the MBDs recruited by methylated DNA that contributes to gene silencing (Amir et al., 1999; Collins et al., 2004).”
• A model for neural development and treatment of Rett Syndrome using human induced pluripotent stem cells (2010, background): “WT allele can be as harmful as a loss of expression (Collins et al., 2004; Ramocki et al., 2009; Van Esch et al., 2005).”
• Neuronal MeCP2 is expressed at near histone-octamer levels and globally alters the chromatin state (2010, background): “For example, expression of MeCP2 from transgenes in mice only brings about phenotypic rescue if the level of protein is close to that in wildtype brain; overexpression by 2-fold is severely detrimental (Collins et al., 2004; Luikenhuis et al., 2004).”
• Brain-specific phosphorylation of MeCP2 regulates activity-dependent Bdnf transcription, dendritic growth, and spine maturation. (2006, background): “In addition, transgenic mice overexpressing wild-type MeCP2 protein also develop a RTT-like phenotype, suggesting that a precise level of MeCP2 expression is critical for proper brain development (Collins et al., 2004; Luikenhuis et al., 2004).”
• Rett Syndrome and Fragile X Syndrome: Different Etiology With Common Molecular Dysfunctions (2021, background): “Additionally, Mecp2 mutant models can also be generated for studying MeCP2 overexpression or MECP2 duplication syndrome (Collins et al., 2004; Na et al., 2012; Bodda et al., 2013).”
• Epigenetic mechanisms in memory formation (2005, background): “Using animal models, it was discovered that overexpression of MECP2 enhanced long-term memory formation and the induction of hippocampal LTP, indicating that MECP2 modulates memory formation and the induction of synaptic plasticit”