Re-establishing ataxin-2 downregulates translation of mutant ataxin-3 and alleviates Machado–Joseph disease

Genetic ablation of ataxin-2 increases several global translation factors in their transcript abundance but decreases translation rate

(2015) M. Fittschen et al.   NEUROGENETICS

Ataxin-2 Regulates RGS8 Translation in a New BAC-SCA2 Transgenic Mouse Model

(2015) Warunee Dansithong et al.   PLoS Genetics

Poly(A)-binding proteins: Structure, domain organization, and activity regulation

(2014) I. A. Eliseeva et al.   BIOCHEMISTRY-MOSCOW

Direct Binding of Ataxin-2 to Distinct Elements in 3′ UTRs Promotes mRNA Stability and Protein Expression

(2014) Moe Yokoshi et al.   MOLECULAR CELL

Caffeine and adenosine A2Areceptor inactivation decrease striatal neuropathology in a lentiviral-based model of Machado-Joseph disease

(2013) Nélio Gonçalves et al.   ANNALS OF NEUROLOGY

Beclin 1 mitigates motor and neuropathological deficits in genetic mouse models of Machado–Joseph disease

(2013) Isabel Nascimento-Ferreira et al.   BRAIN

Ataxin-2 Modulates the Levels of Grb2 and Src but Not Ras Signaling

(2013) Jessica Drost et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

Silencing Mutant Ataxin-3 Rescues Motor Deficits and Neuropathology in Machado-Joseph Disease Transgenic Mice

(2013) Clévio Nóbrega et al.   PLoS One

FMRP and Ataxin-2 function together in long-term olfactory habituation and neuronal translational control

(2013) I. P. Sudhakaran et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A Role for Drosophila ATX2 in Activation of PER Translation and Circadian Behavior

(2013) Y. Zhang et al.   SCIENCE

Calpastatin-mediated inhibition of calpains in the mouse brain prevents mutant ataxin 3 proteolysis, nuclear localization and aggregation, relieving Machado-Joseph disease

(2012) A. T. Simoes et al.   BRAIN

Overexpression of Mutant Ataxin-3 in Mouse Cerebellum Induces Ataxia and Cerebellar Neuropathology

(2012) Clévio Nóbrega et al.   CEREBELLUM

Spinocerebellar Ataxia Type 2: Clinical Presentation, Molecular Mechanisms, and Therapeutic Perspectives

(2012) J. J. Magaña et al.   MOLECULAR NEUROBIOLOGY

Ataxin-2-Like Is a Regulator of Stress Granules and Processing Bodies

(2012) Christian Kaehler et al.   PLoS One

Translational inhibition by deadenylation-independent mechanisms is central to microRNA-mediated silencing in zebrafish

(2012) Y. Mishima et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates Machado–Joseph disease

(2011) Isabel Nascimento-Ferreira et al.   BRAIN

Local RNA Translation at the Synapse and in Disease

(2011) L. Liu-Yesucevitz et al.   JOURNAL OF NEUROSCIENCE

The Ataxin-2 protein is required for microRNA function and synapse-specific long-term olfactory habituation

(2011) C. McCann et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Polyglutamine diseases: The special case of ataxin-3 and Machado–Joseph disease

(2011) Carlos A. Matos et al.   PROGRESS IN NEUROBIOLOGY

Two PABPC1-binding sites in GW182 proteins promote miRNA-mediated gene silencing

(2010) Eric Huntzinger et al.   EMBO JOURNAL

Silencing ataxin-3 mitigates degeneration in a rat model of Machado–Joseph disease: no role for wild-type ataxin-3?

(2010) Sandro Alves et al.   HUMAN MOLECULAR GENETICS

Structural Basis of Binding of P-body-associated Proteins GW182 and Ataxin-2 by the Mlle Domain of Poly(A)-binding Protein

(2010) Guennadi Kozlov et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

(2010) Andrew C. Elden et al.   NATURE

New insights into the pathoanatomy of spinocerebellar ataxia type 3 (Machado–Joseph disease)

(2009) Udo Rüb et al.   CURRENT OPINION IN NEUROLOGY

The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies

(2009) Peter O. Bauer et al.   JOURNAL OF NEUROCHEMISTRY

SUnSET, a nonradioactive method to monitor protein synthesis

(2009) Enrico K Schmidt et al.   NATURE METHODS

Lentivector-mediated rescue from cerebellar ataxia in a mouse model of spinocerebellar ataxia

(2008) Takashi Torashima et al.   EMBO REPORTS

Ataxin-2 associates with rough endoplasmic reticulum

(2008) Simone van de Loo et al.   EXPERIMENTAL NEUROLOGY

Striatal and nigral pathology in a lentiviral rat model of Machado-Joseph disease

(2008) Sandro Alves et al.   HUMAN MOLECULAR GENETICS

Pathogenic Mechanisms of a Polyglutamine-mediated Neurodegenerative Disease, Spinocerebellar Ataxia Type 1

(2008) Huda Y. Zoghbi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Allele-Specific RNA Silencing of Mutant Ataxin-3 Mediates Neuroprotection in a Rat Model of Machado-Joseph Disease

(2008) Sandro Alves et al.   PLoS One

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

(2008) Derek Lessing et al.   PLOS BIOLOGY