PubRank

Ewout R Brunt

Author PubWeight™ 16.17‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Polarised asymmetric inheritance of accumulated protein damage in higher eukaryotes. PLoS Biol 2006 3.06
2 Mutations in the gene PRRT2 cause paroxysmal kinesigenic dyskinesia with infantile convulsions. Cell Rep 2011 2.17
3 In children with Friedreich ataxia, muscle and ataxia parameters are associated. Dev Med Child Neurol 2011 1.45
4 Presence of ATM protein and residual kinase activity correlates with the phenotype in ataxia-telangiectasia: a genotype-phenotype study. Hum Mutat 2012 1.19
5 Accumulation of aberrant ubiquitin induces aggregate formation and cell death in polyglutamine diseases. Hum Mol Genet 2004 1.10
6 Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3. J Neurochem 2004 1.08
7 New insights into the pathoanatomy of spinocerebellar ataxia type 3 (Machado-Joseph disease). Curr Opin Neurol 2008 1.06
8 Degeneration of the cerebellum in Huntington's disease (HD): possible relevance for the clinical picture and potential gateway to pathological mechanisms of the disease process. Brain Pathol 2012 0.92
9 Axonal inclusions in spinocerebellar ataxia type 3. Acta Neuropathol 2010 0.91
10 Spinocerebellar ataxia type 3 (SCA3): thalamic neurodegeneration occurs independently from thalamic ataxin-3 immunopositive neuronal intranuclear inclusions. Brain Pathol 2006 0.91
11 Asian origin for the worldwide-spread mutational event in Machado-Joseph disease. Arch Neurol 2007 0.90
12 Ataxin-2 modulates the levels of Grb2 and SRC but not ras signaling. J Mol Neurosci 2013 0.84
13 Consistent affection of the central somatosensory system in spinocerebellar ataxia type 2 and type 3 and its significance for clinical symptoms and rehabilitative therapy. Brain Res Rev 2006 0.83
14 The International Cooperative Ataxia Rating Scale shows strong age-dependency in children. Dev Med Child Neurol 2009 0.75