David R Lynch

Author PubWeight™ 132.38‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol 2008 21.53
2 Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol 2007 16.81
3 Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children and adolescents. Ann Neurol 2009 9.25
4 Cellular and synaptic mechanisms of anti-NMDA receptor encephalitis. J Neurosci 2010 6.44
5 A patient with encephalitis associated with NMDA receptor antibodies. Nat Clin Pract Neurol 2007 4.63
6 AMPA receptor antibodies in limbic encephalitis alter synaptic receptor location. Ann Neurol 2009 4.49
7 N-methyl-D-aspartate receptor subtypes: multiple roles in excitotoxicity and neurological disease. Neuroscientist 2005 3.11
8 Mutations in the gene PRRT2 cause paroxysmal kinesigenic dyskinesia with infantile convulsions. Cell Rep 2011 2.17
9 ADCK3, an ancestral kinase, is mutated in a form of recessive ataxia associated with coenzyme Q10 deficiency. Am J Hum Genet 2008 2.04
10 The E46K mutation in alpha-synuclein increases amyloid fibril formation. J Biol Chem 2005 1.98
11 Fyn-mediated phosphorylation of NR2B Tyr-1336 controls calpain-mediated NR2B cleavage in neurons and heterologous systems. J Biol Chem 2007 1.84
12 FXN methylation predicts expression and clinical outcome in Friedreich ataxia. Ann Neurol 2012 1.83
13 Anti-NMDA receptor encephalitis antibody binding is dependent on amino acid identity of a small region within the GluN1 amino terminal domain. J Neurosci 2012 1.82
14 Functional consequences of alpha-synuclein tyrosine nitration: diminished binding to lipid vesicles and increased fibril formation. J Biol Chem 2004 1.58
15 Effects of a functional COMT polymorphism on prefrontal cognitive function in patients with 22q11.2 deletion syndrome. Am J Psychiatry 2004 1.43
16 Cytosolic catechols inhibit alpha-synuclein aggregation and facilitate the formation of intracellular soluble oligomeric intermediates. J Neurosci 2006 1.43
17 Clinical measures of dysarthria in Friedreich Ataxia. Mov Disord 2010 1.43
18 Human immunodeficiency virus (HIV)-induced neurotoxicity: roles for the NMDA receptor subtypes. J Neurosci 2006 1.39
19 High-throughput immunoassay for the biochemical diagnosis of Friedreich ataxia in dried blood spots and whole blood. Clin Chem 2013 1.39
20 Polymicrogyria and deletion 22q11.2 syndrome: window to the etiology of a common cortical malformation. Am J Med Genet A 2006 1.38
21 Calpain and synaptic function. Mol Neurobiol 2006 1.37
22 Hemizygous mutations in SNAP29 unmask autosomal recessive conditions and contribute to atypical findings in patients with 22q11.2DS. J Med Genet 2012 1.33
23 Mortality in Friedreich ataxia. J Neurol Sci 2011 1.33
24 A rapid, noninvasive immunoassay for frataxin: utility in assessment of Friedreich ataxia. Mol Genet Metab 2010 1.32
25 Measuring the rate of progression in Friedreich ataxia: implications for clinical trial design. Mov Disord 2010 1.29
26 Selective activation induced cleavage of the NR2B subunit by calpain. J Neurosci 2003 1.26
27 Distinct cleavage patterns of normal and pathologic forms of alpha-synuclein by calpain I in vitro. J Neurochem 2003 1.22
28 Senataxin, the yeast Sen1p orthologue: characterization of a unique protein in which recessive mutations cause ataxia and dominant mutations cause motor neuron disease. Neurobiol Dis 2006 1.21
29 N-methyl-D-aspartate receptor subtype mediated bidirectional control of p38 mitogen-activated protein kinase. J Biol Chem 2005 1.16
30 Cleavage of alpha-synuclein by calpain: potential role in degradation of fibrillized and nitrated species of alpha-synuclein. Biochemistry 2005 1.16
31 Interactions of postsynaptic density-95 and the NMDA receptor 2 subunit control calpain-mediated cleavage of the NMDA receptor. J Neurosci 2004 1.06
32 Proteolysis of the N-methyl-d-aspartate receptor by calpain in situ. J Pharmacol Exp Ther 2002 1.06
33 Advancements in the pathophysiology of Friedreich's Ataxia and new prospects for treatments. Mol Genet Metab 2007 1.04
34 Idebenone in Friedreich ataxia cardiomyopathy-results from a 6-month phase III study (IONIA). Am Heart J 2011 1.01
35 Increased prevalence of unprovoked seizures in patients with a 22q11.2 deletion. Am J Med Genet A 2004 1.00
36 Genetic and clinical heterogeneity in eIF2B-related disorder. J Child Neurol 2008 0.98
37 Apelin, an endogenous neuronal peptide, protects hippocampal neurons against excitotoxic injury. J Neurochem 2007 0.97
38 Blood cells from Friedreich ataxia patients harbor frataxin deficiency without a loss of mitochondrial function. Mitochondrion 2010 0.96
39 Antioxidant use in Friedreich ataxia. J Neurol Sci 2007 0.95
40 A gene expression phenotype in lymphocytes from Friedreich ataxia patients. Ann Neurol 2011 0.94
41 Analysis of echocardiograms in a large heterogeneous cohort of patients with friedreich ataxia. Am J Cardiol 2011 0.94
42 Pharmacotherapy for Friedreich ataxia. CNS Drugs 2009 0.94
43 Effects of COMT genotype on behavioral symptomatology in the 22q11.2 Deletion Syndrome. Child Neuropsychol 2005 0.94
44 Assessment of neurological efficacy of idebenone in pediatric patients with Friedreich's ataxia: data from a 6-month controlled study followed by a 12-month open-label extension study. J Neurol 2011 0.93
45 Axonal α7 nicotinic ACh receptors modulate presynaptic NMDA receptor expression and structural plasticity of glutamatergic presynaptic boutons. Proc Natl Acad Sci U S A 2010 0.93
46 Therapeutic approaches for the treatment of Friedreich's ataxia. Expert Rev Neurother 2014 0.91
47 miR-886-3p levels are elevated in Friedreich ataxia. J Neurosci 2012 0.89
48 N-methyl-D-aspartate receptor mechanosensitivity is governed by C terminus of NR2B subunit. J Biol Chem 2011 0.89
49 Friedreich ataxia clinical outcome measures: natural history evaluation in 410 participants. J Child Neurol 2012 0.89
50 Development of frataxin gene expression measures for the evaluation of experimental treatments in Friedreich's ataxia. PLoS One 2013 0.89
51 Health related quality of life measures in Friedreich Ataxia. J Neurol Sci 2008 0.87
52 Human platelets as a platform to monitor metabolic biomarkers using stable isotopes and LC-MS. Bioanalysis 2013 0.87
53 Novel, complex interruptions of the GAA repeat in small, expanded alleles of two affected siblings with late-onset Friedreich ataxia. Mov Disord 2008 0.86
54 Developmental and cell-selective variations in N-methyl-D-aspartate receptor degradation by calpain. J Neurochem 2006 0.86
55 N-methyl-D-aspartate receptor-dependent regulation of the glutamate transporter excitatory amino acid carrier 1. J Biol Chem 2007 0.86
56 NMDA receptor modulation by the neuropeptide apelin: implications for excitotoxic injury. J Neurochem 2011 0.86
57 Subjective improvement in proprioception in 2 patients with atypical Friedreich ataxia treated with varenicline (Chantix). J Clin Neuromuscul Dis 2009 0.85
58 Calpain and the glutamatergic synapse. Front Biosci (Schol Ed) 2009 0.84
59 Urinary isoprostanes in Friedreich ataxia: lack of correlation with disease features. Mov Disord 2008 0.84
60 Rating disease progression of Friedreich's ataxia by the International Cooperative Ataxia Rating Scale: analysis of a 603-patient database. Brain 2013 0.84
61 Analysis of the visual system in Friedreich ataxia. J Neurol 2013 0.83
62 Health-related quality of life in children with Friedreich ataxia. Pediatr Neurol 2010 0.82
63 Childhood ataxia: clinical features, pathogenesis, key unanswered questions, and future directions. J Child Neurol 2012 0.81
64 Clinical data and characterization of the liver conditional mouse model exclude neoplasia as a non-neurological manifestation associated with Friedreich's ataxia. Dis Model Mech 2012 0.79
65 Longitudinal strain in Friedreich Ataxia: a potential marker for early left ventricular dysfunction. Echocardiography 2013 0.79
66 Glutamatergic autoencephalitides: an emerging field. J Neural Transm (Vienna) 2014 0.78
67 E2F1 in neurons is cleaved by calpain in an NMDA receptor-dependent manner in a model of HIV-induced neurotoxicity. J Neurochem 2014 0.78
68 Mortality in Friedreich's Ataxia. Tex Heart Inst J 2007 0.77
69 Elevation of serum cardiac troponin I in a cross-sectional cohort of asymptomatic subjects with Friedreich ataxia. Int J Cardiol 2012 0.77
70 Pregnancy with Friedreich ataxia: a retrospective review of medical risks and psychosocial implications. Am J Obstet Gynecol 2010 0.76
71 Clinical monitoring in a patient with Friedreich ataxia and osteogenic sarcoma. J Child Neurol 2012 0.75
72 Neuroimmunology: Treatment of anti-NMDA receptor encephalitis--time to be bold? Nat Rev Neurol 2013 0.75
73 Novel diagnostic paradigms for Friedreich ataxia. J Child Neurol 2012 0.75
74 Cross-sectional analysis of electrocardiograms in a large heterogeneous cohort of Friedreich ataxia subjects. J Child Neurol 2012 0.75
75 Juvenile dentatorubral-pallidoluysian atrophy: new clinical features. Pediatr Neurol 2002 0.75
76 Cardiac dysfunction exacerbated by endocrinopathies in Friedreich ataxia: a case series. J Child Neurol 2012 0.75