Ataxin-3 phosphorylation decreases neuronal defects in spinocerebellar ataxia type 3 models

Examination of Ataxin-3 (atx-3) Aggregation by Structural Mass Spectrometry Techniques: A Rationale for Expedited Aggregation upon Polyglutamine (polyQ) Expansion

(2015) Charlotte A. Scarff et al.   MOLECULAR & CELLULAR PROTEOMICS

Dominant negative effect of polyglutamine expansion perturbs normal function of ataxin-3 in neuronal cells

(2014) Andreia Neves-Carvalho et al.   HUMAN MOLECULAR GENETICS

Deciphering key features in protein structures with the new ENDscript server

(2014) Xavier Robert et al.   NUCLEIC ACIDS RESEARCH

Mutant Ataxin-3 with an Abnormally Expanded Polyglutamine Chain Disrupts Dendritic Development and Metabotropic Glutamate Receptor Signaling in Mouse Cerebellar Purkinje Cells

(2013) Ayumu Konno et al.   CEREBELLUM

Widespread neuronal damage and cognitive dysfunction in spinocerebellar ataxia type 3

(2013) Tátila Martins Lopes et al.   JOURNAL OF NEUROLOGY

Nonmotor and extracerebellar features in Machado-Joseph disease: A review

(2013) José Luiz Pedroso et al.   MOVEMENT DISORDERS

Clinical features, neurogenetics and neuropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7

(2013) Udo Rüb et al.   PROGRESS IN NEUROBIOLOGY

Trinucleotide Repeats: A Structural Perspective

(2013) Bruno Almeida et al.   Frontiers in Neurology

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

Fiji: an open-source platform for biological-image analysis

(2012) Johannes Schindelin et al.   NATURE METHODS

Phosphorylation-dependent activity of the deubiquitinase DUBA

(2012) Oscar W Huang et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Phosphorylation Meets Proteolysis

(2012) Martin Renatus et al.   STRUCTURE

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

(2011) Isabel Nascimento-Ferreira et al.   BRAIN

Neocortical Atrophy in Machado-Joseph Disease: A Longitudinal Neuroimaging Study

(2011) Anelyssa D’Abreu et al.   JOURNAL OF NEUROIMAGING

Early Changes in Cerebellar Physiology Accompany Motor Dysfunction in the Polyglutamine Disease Spinocerebellar Ataxia Type 3

(2011) V. G. Shakkottai et al.   JOURNAL OF NEUROSCIENCE

Excitation-induced ataxin-3 aggregation in neurons from patients with Machado–Joseph disease

(2011) Philipp Koch et al.   NATURE

Machado-Joseph Disease: from first descriptions to new perspectives

(2011) Conceição Bettencourt et al.   Orphanet Journal of Rare Diseases

Pathogenic polyglutamine proteins cause dendrite defects associated with specific actin cytoskeletal alterations in Drosophila

(2011) S. B. Lee 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

Absence of ataxin-3 leads to cytoskeletal disorganization and increased cell death

(2010) Ana-João Rodrigues et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Functional interactions as a survival strategy against abnormal aggregation

(2010) Masino Laura et al.   FASEB 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

Crystal Structure of a Josephin-Ubiquitin Complex

(2010) Stephen D. Weeks et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Activity and Cellular Functions of the Deubiquitinating Enzyme and Polyglutamine Disease Protein Ataxin-3 Are Regulated by Ubiquitination at Lysine 117

(2010) Sokol V. Todi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Repeat expansion disease: progress and puzzles in disease pathogenesis

(2010) Albert R. La Spada et al.   NATURE REVIEWS GENETICS

Structural Transformation of the Tandem Ubiquitin-Interacting Motifs in Ataxin-3 and Their Cooperative Interactions with Ubiquitin Chains

(2010) Ai-Xin Song et al.   PLoS One

Splice Isoforms of the Polyglutamine Disease Protein Ataxin-3 Exhibit Similar Enzymatic yet Different Aggregation Properties

(2010) Ginny Marie Harris et al.   PLoS One

Polyglutamine Diseases: Where does Toxicity Come from? What is Toxicity? Where are We Going?

(2010) T. Takahashi et al.   Journal of Molecular Cell Biology

The Chemical Biology of Protein Phosphorylation

(2009) Mary Katherine Tarrant et al.   Annual Review of Biochemistry

Josephin domain of ataxin-3 contains two distinct ubiquitin-binding sites

(2009) Giuseppe Nicastro et al.   BIOPOLYMERS

Post-translational modifications of expanded polyglutamine proteins: impact on neurotoxicity

(2009) M. Pennuto et al.   HUMAN MOLECULAR GENETICS

CK2-dependent phosphorylation determines cellular localization and stability of ataxin-3

(2009) Thorsten Mueller et al.   HUMAN MOLECULAR GENETICS

Phosphorylation of Threonine 3

(2009) Charity T. Aiken et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies

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

Breaking the chains: structure and function of the deubiquitinases

(2009) David Komander et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

A transgenic mouse model of spinocerebellar ataxia type 3 resembling late disease onset and gender-specific instability of CAG repeats

(2009) Jana Boy et al.   NEUROBIOLOGY OF DISEASE

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

(2008) Sandro Alves et al.   HUMAN MOLECULAR GENETICS

Polyglutamine-expanded ataxin-3 causes cerebellar dysfunction of SCA3 transgenic mice by inducing transcriptional dysregulation

(2008) An-Hsun Chou et al.   NEUROBIOLOGY OF DISEASE

Study of subcellular localization and proteolysis of ataxin-3

(2008) Chiara Pozzi et al.   NEUROBIOLOGY OF DISEASE

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

Casein kinase 2 interacts with and phosphorylates ataxin-3

(2008) Rui-Song Tao et al.   Neuroscience Bulletin