Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS

Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis

2006 • 5,928 citations

From charcot to lou gehrig: deciphering selective motor neuron death in als

2001 • 1,373 citations

Trinucleotide Repeat Disorders

2007 • 1,341 citations

α-Synuclein Blocks ER-Golgi Traffic and Rab1 Rescues Neuron Loss in Parkinson's Models

2006 • 1,328 citations

Chaperone Suppression of α-Synuclein Toxicity in a Drosophila Model for Parkinson's Disease

2002 • 1,220 citations

Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2

1996 • 1,081 citations

Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70

1999 • 830 citations

Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats

1996 • 824 citations

Yeast Cells Provide Insight into Alpha-Synuclein Biology and Pathobiology

2003 • 790 citations

Identification of the spinocerebellar ataxia type 2 gene using a direct identification of repeat expansion and cloning technique, DIRECT

1996 • 745 citations

TDP-43 Is Intrinsically Aggregation-prone, and Amyotrophic Lateral Sclerosis-linked Mutations Accelerate Aggregation and Increase Toxicity

2009 • 707 citations

Characterization and Functional Implications of the RNA Binding Properties of Nuclear Factor TDP-43, a Novel Splicing Regulator ofCFTR Exon 9

2001 • 635 citations

Disturbance of Nuclear and Cytoplasmic TAR DNA-binding Protein (TDP-43) Induces Disease-like Redistribution, Sequestration, and Aggregate Formation

2008 • 589 citations

Rethinking ALS: The FUS about TDP-43

2009 • 567 citations

Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins

2000 • 549 citations

Misfolded CuZnSOD and amyotrophic lateral sclerosis

2003 • 542 citations

α-Synuclein is part of a diverse and highly conserved interaction network that includes PARK9 and manganese toxicity

2009 • 523 citations

P bodies promote stress granule assembly in Saccharomyces cerevisiae

2008 • 491 citations

TDP‐43 is recruited to stress granules in conditions of oxidative insult

2009 • 484 citations

Global Analysis of TDP-43 Interacting Proteins Reveals Strong Association with RNA Splicing and Translation Machinery

2009 • 466 citations

Eukaryotic Proteasomes Cannot Digest Polyglutamine Sequences and Release Them during Degradation of Polyglutamine-Containing Proteins

2004 • 409 citations

A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDP-43 aggregation and cellular toxicity

2008 • 397 citations

Ataxin-2 Interacts with the DEAD/H-Box RNA Helicase DDX6 and Interferes with P-Bodies and Stress Granules

2006 • 330 citations

RNA toxicity is a component of ataxin-3 degeneration in Drosophila

2008 • 319 citations

Mutations in TDP-43 link glycine-rich domain functions to amyotrophic lateral sclerosis

2009 • 287 citations

Spinocerebellar ataxia 2 (SCA2)

2008 • 188 citations

Pbp1p, a Factor Interacting withSaccharomyces cerevisiaePoly(A)-Binding Protein, Regulates Polyadenylation

1998 • 178 citations

Expansion of the polyQ repeat in ataxin-2 alters its Golgi localization, disrupts the Golgi complex and causes cell death

2003 • 134 citations

Polyglutamine Genes Interact to Modulate the Severity and Progression of Neurodegeneration in Drosophila

2008 • 89 citations

dAtaxin-2 Mediates Expanded Ataxin-1-Induced Neurodegeneration in a Drosophila Model of SCA1

2007 • 89 citations

The expansion of the CAG repeat in ataxin-2 is a frequent cause of autosomal dominant spinocerebellar ataxia

1997 • 74 citations

Spinocerebellar ataxia type 2 with levodopa‐responsive parkinsonism culminating in motor neuron disease

2004 • 52 citations

Rare association of motor neuron disease and spinocerebellar ataxia type 2 (SCA2): a new case and review of the literature

2009 • 51 citations

Therapeutic developments in the treatment of amyotrophic lateral sclerosis

2002 • 24 citations