Rare Diseases Clinical Research Network: Neurophysiological Correlates

Clinical Trial ID NCT03077308

PubWeight™ 0.00‹?›

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Top papers

Rank Title Journal Year PubWeight™‹?›
1 EEGLAB: an open source toolbox for analysis of single-trial EEG dynamics including independent component analysis. J Neurosci Methods 2004 34.47
2 MeCP2 controls excitatory synaptic strength by regulating glutamatergic synapse number. Neuron 2007 4.15
3 Specific mutations in methyl-CpG-binding protein 2 confer different severity in Rett syndrome. Neurology 2008 3.74
4 Investigating genotype-phenotype relationships in Rett syndrome using an international data set. Neurology 2008 2.10
5 A critical and cell-autonomous role for MeCP2 in synaptic scaling up. J Neurosci 2012 1.69
6 Methyl-CpG-binding protein 2 (MECP2) mutation type is associated with disease severity in Rett syndrome. J Med Genet 2014 1.58
7 Validating γ oscillations and delayed auditory responses as translational biomarkers of autism. Biol Psychiatry 2010 1.58
8 Rett syndrome mutation MeCP2 T158A disrupts DNA binding, protein stability and ERP responses. Nat Neurosci 2011 1.53
9 Neurophysiologic evaluation of auditory recognition memory in healthy newborn infants and infants of diabetic mothers. J Pediatr 2000 1.51
10 Safety, pharmacokinetics, and preliminary assessment of efficacy of mecasermin (recombinant human IGF-1) for the treatment of Rett syndrome. Proc Natl Acad Sci U S A 2014 1.40
11 Bdnf overexpression in hippocampal neurons prevents dendritic atrophy caused by Rett-associated MECP2 mutations. Neurobiol Dis 2009 1.31
12 The impact of MeCP2 loss- or gain-of-function on synaptic plasticity. Neuropsychopharmacology 2012 1.22
13 A mouse model for MeCP2 duplication syndrome: MeCP2 overexpression impairs learning and memory and synaptic transmission. J Neurosci 2012 1.18
14 MeCP2 expression and function during brain development: implications for Rett syndrome's pathogenesis and clinical evolution. Brain Dev 2005 1.16
15 Transient and steady-state auditory gamma-band responses in first-degree relatives of people with autism spectrum disorder. Mol Autism 2011 1.13
16 Alterations of cortical and hippocampal EEG activity in MeCP2-deficient mice. Neurobiol Dis 2010 1.08
17 Loss of CDKL5 disrupts kinome profile and event-related potentials leading to autistic-like phenotypes in mice. Proc Natl Acad Sci U S A 2012 1.02
18 MeCP2 mutation results in compartment-specific reductions in dendritic branching and spine density in layer 5 motor cortical neurons of YFP-H mice. PLoS One 2012 0.97
19 Visual evoked potentials detect cortical processing deficits in Rett syndrome. Ann Neurol 2015 0.96
20 Comparison of novel computer detectors and human performance for spike detection in intracranial EEG. Clin Neurophysiol 2007 0.92
21 MeCP2+/- mouse model of RTT reproduces auditory phenotypes associated with Rett syndrome and replicate select EEG endophenotypes of autism spectrum disorder. Neurobiol Dis 2012 0.91
22 Reduced seizure threshold and altered network oscillatory properties in a mouse model of Rett syndrome. Neuroscience 2012 0.90
23 Auditory brainstem response findings in Rett syndrome: stability over time. J Pediatr 2000 0.82
24 Induced gamma oscillations differentiate familiar and novel voices in children with MECP2 duplication and Rett syndromes. J Child Neurol 2014 0.79
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