PubRank

Wim Robberecht

Author PubWeight™ 151.70‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Progressive multifocal leukoencephalopathy after natalizumab therapy for Crohn's disease. N Engl J Med 2005 6.33
2 A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study. Lancet Neurol 2011 5.01
3 Analysis of immune-related loci identifies 48 new susceptibility variants for multiple sclerosis. Nat Genet 2013 4.62
4 Genome-wide association study identifies 19p13.3 (UNC13A) and 9p21.2 as susceptibility loci for sporadic amyotrophic lateral sclerosis. Nat Genet 2009 4.38
5 VEGF is a modifier of amyotrophic lateral sclerosis in mice and humans and protects motoneurons against ischemic death. Nat Genet 2003 4.19
6 Chromosome 9p21 in sporadic amyotrophic lateral sclerosis in the UK and seven other countries: a genome-wide association study. Lancet Neurol 2010 3.61
7 Treatment of motoneuron degeneration by intracerebroventricular delivery of VEGF in a rat model of ALS. Nat Neurosci 2004 3.31
8 Disrupted function and axonal distribution of mutant tyrosyl-tRNA synthetase in dominant intermediate Charcot-Marie-Tooth neuropathy. Nat Genet 2006 3.04
9 Mutant small heat-shock protein 27 causes axonal Charcot-Marie-Tooth disease and distal hereditary motor neuropathy. Nat Genet 2004 3.00
10 Progranulin functions as a neurotrophic factor to regulate neurite outgrowth and enhance neuronal survival. J Cell Biol 2008 2.91
11 Hot-spot residue in small heat-shock protein 22 causes distal motor neuropathy. Nat Genet 2004 2.86
12 Serum biomarker for progranulin-associated frontotemporal lobar degeneration. Ann Neurol 2009 2.83
13 ITPR2 as a susceptibility gene in sporadic amyotrophic lateral sclerosis: a genome-wide association study. Lancet Neurol 2007 2.80
14 MFN2 mutation distribution and genotype/phenotype correlation in Charcot-Marie-Tooth type 2. Brain 2006 2.77
15 A yeast functional screen predicts new candidate ALS disease genes. Proc Natl Acad Sci U S A 2011 2.68
16 EPHA4 is a disease modifier of amyotrophic lateral sclerosis in animal models and in humans. Nat Med 2012 2.65
17 Genetic variation in DPP6 is associated with susceptibility to amyotrophic lateral sclerosis. Nat Genet 2007 2.63
18 HDAC6 inhibitors reverse axonal loss in a mouse model of mutant HSPB1-induced Charcot-Marie-Tooth disease. Nat Med 2011 2.60
19 Neuroinflammation in amyotrophic lateral sclerosis: role of glial activation in motor neuron disease. Lancet Neurol 2011 2.34
20 Guidelines for preclinical animal research in ALS/MND: A consensus meeting. Amyotroph Lateral Scler 2010 2.14
21 ELP3 controls active zone morphology by acetylating the ELKS family member Bruchpilot. Neuron 2011 1.83
22 Oligodendrocyte dysfunction in the pathogenesis of amyotrophic lateral sclerosis. Brain 2013 1.78
23 The C9ORF72 expansion mutation is a common cause of ALS+/-FTD in Europe and has a single founder. Eur J Hum Genet 2012 1.71
24 Genome-wide association analyses in Han Chinese identify two new susceptibility loci for amyotrophic lateral sclerosis. Nat Genet 2013 1.57
25 Variants of the elongator protein 3 (ELP3) gene are associated with motor neuron degeneration. Hum Mol Genet 2008 1.46
26 Quantitative diffusion tensor imaging in amyotrophic lateral sclerosis. Neuroimage 2006 1.45
27 Angiogenin variants in Parkinson disease and amyotrophic lateral sclerosis. Ann Neurol 2011 1.45
28 Astrocytes regulate GluR2 expression in motor neurons and their vulnerability to excitotoxicity. Proc Natl Acad Sci U S A 2007 1.42
29 Minocycline delays disease onset and mortality in a transgenic model of ALS. Neuroreport 2002 1.40
30 Unilateral white matter involvement in Krabbe disease. Arch Neurol 2011 1.38
31 Influence of arterial input function on hypoperfusion volumes measured with perfusion-weighted imaging. Stroke 2003 1.33
32 Neuronal inclusion protein TDP-43 has no primary genetic role in FTD and ALS. Neurobiol Aging 2008 1.31
33 Ablation of proliferating microglia does not affect motor neuron degeneration in amyotrophic lateral sclerosis caused by mutant superoxide dismutase. J Neurosci 2008 1.27
34 Guidelines for the preclinical in vivo evaluation of pharmacological active drugs for ALS/MND: report on the 142nd ENMC international workshop. Amyotroph Lateral Scler 2007 1.27
35 Progranulin is neurotrophic in vivo and protects against a mutant TDP-43 induced axonopathy. PLoS One 2010 1.25
36 Novel role for vascular endothelial growth factor (VEGF) receptor-1 and its ligand VEGF-B in motor neuron degeneration. J Neurosci 2008 1.23
37 Variant on 9p21 strongly associates with coronary heart disease, but lacks association with common stroke. Eur J Hum Genet 2009 1.22
38 Relative contribution of mutations in genes for autosomal dominant distal hereditary motor neuropathies: a genotype-phenotype correlation study. Brain 2008 1.21
39 Efficacy and safety of xaliproden in amyotrophic lateral sclerosis: results of two phase III trials. Amyotroph Lateral Scler Other Motor Neuron Disord 2004 1.15
40 RNA metabolism and the pathogenesis of motor neuron diseases. Trends Neurosci 2010 1.15
41 Upper and extra-motoneuron involvement in early motoneuron disease: a diffusion tensor imaging study. Brain 2011 1.13
42 Effects of vascular endothelial growth factor (VEGF) on motor neuron degeneration. Neurobiol Dis 2004 1.09
43 Overexpression of mutant superoxide dismutase 1 causes a motor axonopathy in the zebrafish. Hum Mol Genet 2007 1.08
44 Quantitative diffusion tensor imaging in amyotrophic lateral sclerosis: revisited. Hum Brain Mapp 2009 1.06
45 Recent advances in motor neuron disease. Curr Opin Neurol 2009 1.05
46 Upregulation of HSP27 in a transgenic model of ALS. J Neuropathol Exp Neurol 2002 1.05
47 GluR2 deficiency accelerates motor neuron degeneration in a mouse model of amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 2005 1.04
48 The AMPA receptor antagonist NBQX prolongs survival in a transgenic mouse model of amyotrophic lateral sclerosis. Neurosci Lett 2003 1.03
49 The neurobiology of amyotrophic lateral sclerosis. Eur J Neurosci 2010 1.02
50 Age of onset of amyotrophic lateral sclerosis is modulated by a locus on 1p34.1. Neurobiol Aging 2012 1.02
51 Pathogenic cysteine mutations affect progranulin function and production of mature granulins. J Neurochem 2009 1.01
52 Inhibition of p38 mitogen activated protein kinase activation and mutant SOD1(G93A)-induced motor neuron death. Neurobiol Dis 2007 1.00
53 Efficacy and safety of Privigen(®) in patients with chronic inflammatory demyelinating polyneuropathy: results of a prospective, single-arm, open-label Phase III study (the PRIMA study). J Peripher Nerv Syst 2013 1.00
54 VEGF protects motor neurons against excitotoxicity by upregulation of GluR2. Neurobiol Aging 2009 0.99
55 A genome-wide association meta-analysis identifies a novel locus at 17q11.2 associated with sporadic amyotrophic lateral sclerosis. Hum Mol Genet 2013 0.98
56 Skeletal muscle properties in a transgenic mouse model for amyotrophic lateral sclerosis: effects of creatine treatment. Neurobiol Dis 2003 0.98
57 Hdac6 deletion delays disease progression in the SOD1G93A mouse model of ALS. Hum Mol Genet 2013 0.97
58 NIPA1 polyalanine repeat expansions are associated with amyotrophic lateral sclerosis. Hum Mol Genet 2012 0.97
59 Benefits of intensive insulin therapy on neuromuscular complications in routine daily critical care practice: a retrospective study. Crit Care 2009 0.96
60 Glatiramer acetate has no impact on disease progression in ALS at 40 mg/day: a double- blind, randomized, multicentre, placebo-controlled trial. Amyotroph Lateral Scler 2009 0.96
61 Mapping of gene expression reveals CYP27A1 as a susceptibility gene for sporadic ALS. PLoS One 2012 0.95
62 UNC13A is a modifier of survival in amyotrophic lateral sclerosis. Neurobiol Aging 2011 0.95
63 The neurotrophic properties of progranulin depend on the granulin E domain but do not require sortilin binding. Neurobiol Aging 2013 0.94
64 Benefit of the Awaji diagnostic algorithm for amyotrophic lateral sclerosis: a prospective study. Ann Neurol 2011 0.94
65 Clinical implications of recent breakthroughs in amyotrophic lateral sclerosis. Curr Opin Neurol 2013 0.92
66 A large genome scan for rare CNVs in amyotrophic lateral sclerosis. Hum Mol Genet 2010 0.92
67 The G93C mutation in superoxide dismutase 1: clinicopathologic phenotype and prognosis. Arch Neurol 2006 0.91
68 Over-expression of Hsp27 does not influence disease in the mutant SOD1(G93A) mouse model of amyotrophic lateral sclerosis. J Neurochem 2008 0.91
69 Multicentre quality control evaluation of different biomarker candidates for amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2014 0.90
70 Microglial upregulation of progranulin as a marker of motor neuron degeneration. J Neuropathol Exp Neurol 2010 0.89
71 Novel COL4A1 mutations cause cerebral small vessel disease by haploinsufficiency. Hum Mol Genet 2012 0.87
72 Heterogeneity in motoneuron disease. Trends Neurosci 2007 0.87
73 H63D polymorphism in HFE is not associated with amyotrophic lateral sclerosis. Neurobiol Aging 2012 0.87
74 Whole-genome sequencing reveals a coding non-pathogenic variant tagging a non-coding pathogenic hexanucleotide repeat expansion in C9orf72 as cause of amyotrophic lateral sclerosis. Hum Mol Genet 2012 0.86
75 VEGF modulates NMDA receptors activity in cerebellar granule cells through Src-family kinases before synapse formation. Proc Natl Acad Sci U S A 2011 0.86
76 Rapamycin increases survival in ALS mice lacking mature lymphocytes. Mol Neurodegener 2013 0.86
77 Modifying expression of EphA4 and its downstream targets improves functional recovery after stroke. Hum Mol Genet 2013 0.85
78 Mutant HSPB8 causes motor neuron-specific neurite degeneration. Hum Mol Genet 2010 0.85
79 Role of mitochondria in kainate-induced fast Ca2+ transients in cultured spinal motor neurons. Cell Calcium 2007 0.84
80 Rare mutations in SQSTM1 modify susceptibility to frontotemporal lobar degeneration. Acta Neuropathol 2014 0.84
81 TNFRSF1A coding variants in multiple sclerosis. J Neuroimmunol 2011 0.83
82 Accuracy of diffusion-weighted MR imaging in the diagnosis of sporadic Creutzfeldt-Jakob disease. J Neurol 2003 0.83
83 Cardiac involvement and CTG expansion in myotonic dystrophy. J Neurol 2002 0.83
84 Genetic variant in the HSPB1 promoter region impairs the HSP27 stress response. Hum Mutat 2007 0.83
85 Non-invasive ventilation in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2013 0.83
86 C9orf72 and UNC13A are shared risk loci for amyotrophic lateral sclerosis and frontotemporal dementia: a genome-wide meta-analysis. Ann Neurol 2014 0.83
87 Prevention of intestinal obstruction reveals progressive neurodegeneration in mutant TDP-43 (A315T) mice. Mol Neurodegener 2014 0.83
88 Vascular endothelial growth factor in amyotrophic lateral sclerosis and other neurodegenerative diseases. Muscle Nerve 2006 0.83
89 Frequency of C9orf72 repeat expansions in amyotrophic lateral sclerosis: a Belgian cohort study. Neurobiol Aging 2013 0.82
90 Long-lasting changes in GABA responsiveness in cultured neurons. Neurosci Lett 2004 0.81
91 Role of matrix metalloproteinase-9 in a mouse model for amyotrophic lateral sclerosis. Neuroreport 2005 0.81
92 Ivermectin inhibits AMPA receptor-mediated excitotoxicity in cultured motor neurons and extends the life span of a transgenic mouse model of amyotrophic lateral sclerosis. Neurobiol Dis 2006 0.81
93 Analysis of FGGY as a risk factor for sporadic amyotrophic lateral sclerosis. Amyotroph Lateral Scler 2009 0.81
94 Differentiation of hereditary spastic paraparesis from primary lateral sclerosis in sporadic adult-onset upper motor neuron syndromes. Arch Neurol 2009 0.81
95 Modeling neurodegenerative diseases in zebrafish embryos. Methods Mol Biol 2011 0.80
96 No evidence for shared genetic basis of common variants in multiple sclerosis and amyotrophic lateral sclerosis. Hum Mol Genet 2013 0.80
97 Microglia in amyotrophic lateral sclerosis. Acta Neurol Belg 2007 0.80
98 Manofluorographic evaluation of swallowing in amyotrophic lateral sclerosis and its relationship with clinical evaluation of swallowing. Amyotroph Lateral Scler 2006 0.80
99 The role of oligodendroglial dysfunction in amyotrophic lateral sclerosis. Neurodegener Dis Manag 2014 0.79
100 Neuronal overexpression of IP₃ receptor 2 is detrimental in mutant SOD1 mice. Biochem Biophys Res Commun 2012 0.78
101 Genetic ablation of phospholipase C delta 1 increases survival in SOD1(G93A) mice. Neurobiol Dis 2013 0.78
102 Hypersensitivity pneumonitis possibly caused by riluzole therapy in ALS. Neurology 2003 0.77
103 Progranulin does not affect motor neuron degeneration in mutant SOD1 mice and rats. Neurobiol Aging 2013 0.77
104 CuZn-superoxide dismutase in D90A heterozygotes from recessive and dominant ALS pedigrees. Neurobiol Dis 2002 0.76
105 Autosomal recessive spastic ataxia of Charlevoix-Saguenay. Neurology 2009 0.76
106 Polymorphisms in the GluR2 gene are not associated with amyotrophic lateral sclerosis. Neurobiol Aging 2010 0.76
107 Reverse transcriptase takes ALS back to viruses. Neurology 2005 0.76
108 Developments in treatments for amyotrophic lateral sclerosis via intracerebroventricular or intrathecal delivery. Expert Opin Investig Drugs 2014 0.76
109 Peripheral neuropathy and 46XY gonadal dysgenesis: a heterogeneous entity. Neuromuscul Disord 2009 0.75
110 Amyotrophic lateral sclerosis. Nat Rev Dis Prim 2017 0.75
111 Report from the Italian Ministerial Committee regarding the diagnosis, care and assistance of patients with ALS. Amyotroph Lateral Scler Other Motor Neuron Disord 2005 0.75
112 Synaptopodin and 4 novel genes identified in primary sensory neurons. Mol Cell Neurosci 2005 0.75
113 An unusual presentation of Guillain-Barré syndrome associated with monospecific anti-GD1b antibodies. J Neurol Neurosurg Psychiatry 2010 0.75
114 Chapter 19 Therapies in amyotrophic lateral sclerosis: Options for the near and far future. Handb Clin Neurol 2007 0.75