Roles of Post-translational Modifications in Spinocerebellar Ataxias

Post-Translational Modifications (PTMs), Identified on Endogenous Huntingtin, Cluster within Proteolytic Domains between HEAT Repeats

(2017) Tamara Ratovitski et al.   JOURNAL OF PROTEOME RESEARCH

Polyglutamine expansion of ataxin-3 alters its degree of ubiquitination and phosphorylation at specific sites

(2017) Line V. Kristensen et al.   NEUROCHEMISTRY INTERNATIONAL

FAF1 phosphorylation by AKT accumulates TGF-β type II receptor and drives breast cancer metastasis

(2017) Feng Xie et al.   Nature Communications

Ataxin-1 regulates epithelial–mesenchymal transition of cervical cancer cells

(2017) A-ram Kang et al.   Oncotarget

Protein SUMOylation modification and its associations with disease

(2017) Yanfang Yang et al.   Open Biology

The Truncated C-terminal Fragment of Mutant ATXN3 Disrupts Mitochondria Dynamics in Spinocerebellar Ataxia Type 3 Models

(2017) Jung-Yu Hsu et al.   Frontiers in Molecular Neuroscience

Post-translational Modifications and Protein Quality Control in Motor Neuron and Polyglutamine Diseases

(2017) Fabio Sambataro et al.   Frontiers in Molecular Neuroscience

Ubiquitin-dependent and independent roles of SUMO in proteostasis

(2016) Frauke Liebelt et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

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

(2016) Carlos A. Matos et al.   JOURNAL OF CELL BIOLOGY

Safety and efficacy of valproic acid treatment in SCA3/MJD patients

(2016) Li-Fang Lei et al.   PARKINSONISM & RELATED DISORDERS

SUMOylation of the brain-predominant Ataxin-3 isoform modulates its interaction with p97

(2015) Bruno Almeida et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

A key lysine residue in the AXH domain of ataxin-1 is essential for its ubiquitylation

(2015) A-ram Kang et al.   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

Proteolytic cleavage of ataxin-7 promotes SCA7 retinal degeneration and neurological dysfunction

(2015) S. J. Guyenet et al.   HUMAN MOLECULAR GENETICS

Toward therapeutic targets for SCA3: Insight into the role of Machado–Joseph disease protein ataxin-3 in misfolded proteins clearance

(2015) Xiaoling Li et al.   PROGRESS IN NEUROBIOLOGY

Role of transcription factor acetylation in the regulation of metabolic homeostasis

(2015) Joo-Man Park et al.   Protein & Cell

Autosomal dominant cerebellar ataxias: a systematic review of clinical features

(2014) M. Rossi et al.   EUROPEAN JOURNAL OF NEUROLOGY

Calpain inhibition reduces ataxin-3 cleavage alleviating neuropathology and motor impairments in mouse models of Machado–Joseph disease

(2014) Ana Teresa Simões et al.   HUMAN MOLECULAR GENETICS

The design and delivery of a PKA inhibitory polypeptide to treat SCA1

(2014) Scoty M. Hearst et al.   JOURNAL OF NEUROCHEMISTRY

CDK5 protects from caspase-induced Ataxin-3 cleavage and neurodegeneration

(2014) Jan Liman et al.   JOURNAL OF NEUROCHEMISTRY

A Cellular System that Degrades Misfolded Proteins and Protects against Neurodegeneration

(2014) Lili Guo et al.   MOLECULAR CELL

Beyond the Glutamine Expansion: Influence of Posttranslational Modifications of Ataxin-1 in the Pathogenesis of Spinocerebellar Ataxia Type 1

(2014) Hyoungseok Ju et al.   MOLECULAR NEUROBIOLOGY

KCNC3R420H, a K+ channel mutation causative in spinocerebellar ataxia 13 displays aberrant intracellular trafficking

(2014) Carolina Gallego-Iradi et al.   NEUROBIOLOGY OF DISEASE

Cyclin-dependent kinase 5 phosphorylates and induces the degradation of ataxin-2

(2014) Akiko Asada et al.   NEUROSCIENCE LETTERS

Ubiquitin-binding site 2 of ataxin-3 prevents its proteasomal degradation by interacting with Rad23

(2014) Jessica R. Blount et al.   Nature Communications

Deregulation of the actin cytoskeleton and macropinocytosis in response to phorbol ester by the mutant protein kinase C gamma that causes spinocerebellar ataxia type 14

(2014) Kazuhiro Yamamoto et al.   Frontiers in Physiology

Polyglutamine Disease Toxicity Is Regulated by Nemo-like Kinase in Spinocerebellar Ataxia Type 1

(2013) H. Ju et al.   JOURNAL OF NEUROSCIENCE

Ataxin-3 Protein and RNA Toxicity in Spinocerebellar Ataxia Type 3: Current Insights and Emerging Therapeutic Strategies

(2013) Melvin M. Evers et al.   MOLECULAR NEUROBIOLOGY

Histone deacetylase-3 interacts with ataxin-7 and is altered in a spinocerebellar ataxia type 7 mouse model

(2013) Carlotta E Duncan et al.   Molecular Neurodegeneration

RAS–MAPK–MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1

(2013) Jeehye Park et al.   NATURE

SUMO-1 Modification on K166 of PolyQ-Expanded aTaxin-3 Strengthens Its Stability and Increases Its Cytotoxicity

(2013) Ya-Fang Zhou et al.   PLoS One

Sodium Valproate Alleviates Neurodegeneration in SCA3/MJD via Suppressing Apoptosis and Rescuing the Hypoacetylation Levels of Histone H3 and H4

(2013) Jiping Yi et al.   PLoS One

Identification of CHIP as a Novel Causative Gene for Autosomal Recessive Cerebellar Ataxia

(2013) Yuting Shi et al.   PLoS One

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

Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3)

(2012) J. Hubener et al.   HUMAN MOLECULAR GENETICS

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

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

Function and regulation of SUMO proteases

(2012) Christopher M. Hickey et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

LANP mediates neuritic pathology in Spinocerebellar ataxia type 1

(2012) Marija Cvetanovic et al.   NEUROBIOLOGY OF DISEASE

SCA1—Phosphorylation, a regulator of Ataxin-1 function and pathogenesis

(2012) Harry T. Orr   PROGRESS IN NEUROBIOLOGY

The importance of serine 776 in Ataxin-1 partner selection: A FRET Analysis

(2012) Rajesh P. Menon et al.   Scientific Reports

Polyglutamine (polyQ) disorders

(2012) Dorit Cohen-Carmon et al.   Nucleus

14-3-3 Binding to Ataxin-1(ATXN1) Regulates Its Dephosphorylation at Ser-776 and Transport to the Nucleus

(2011) Shaojuan Lai et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

(2011) Philipp Koch et al.   NATURE

SAHA Decreases HDAC 2 and 4 Levels In Vivo and Improves Molecular Phenotypes in the R6/2 Mouse Model of Huntington's Disease

(2011) Michal Mielcarek et al.   PLoS One

Cell-autonomous and non-cell-autonomous toxicity in polyglutamine diseases

(2011) Fabio Sambataro et al.   PROGRESS IN NEUROBIOLOGY

Oxidative stress-enhanced SUMOylation and aggregation of ataxin-1: Implication of JNK pathway

(2010) Joohyun Ryu et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Apoptosis of hepatic stellate cells mediated by specific protein nitration

(2010) Teresa Mòdol et al.   BIOCHEMICAL PHARMACOLOGY

CK2 and GSK3 phosphorylation on S29 controls wild-type ATXN3 nuclear uptake

(2010) V. Pastori et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

A Tissue-Specific Atlas of Mouse Protein Phosphorylation and Expression

(2010) Edward L. Huttlin et al.   CELL

Multiple faces of the SAGA complex

(2010) Evangelia Koutelou et al.   CURRENT OPINION IN CELL BIOLOGY

Proteolytic processing regulates pathological accumulation in dentatorubral-pallidoluysian atrophy

(2010) Yasuyo Suzuki et al.   FEBS Journal

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

Autosomal dominant cerebellar ataxias: polyglutamine expansions and beyond

(2010) Alexandra Durr   LANCET NEUROLOGY

Molecular susceptibility to glycation and its implication in diabetes mellitus and related diseases

(2010) José D. Méndez et al.   MOLECULAR AND CELLULAR BIOCHEMISTRY

Amyloid precursor-like protein 2 cleavage contributes to neuronal intranuclear inclusions and cytotoxicity in spinocerebellar ataxia-7 (SCA7)

(2010) Junko Takahashi-Fujigasaki et al.   NEUROBIOLOGY OF DISEASE

Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3

(2009) Sokol V Todi et al.   EMBO JOURNAL

SUMOylation attenuates the aggregation propensity and cellular toxicity of the polyglutamine expanded ataxin-7

(2009) Alexandre Janer et al.   HUMAN MOLECULAR GENETICS

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

(2009) Thorsten Mueller et al.   HUMAN MOLECULAR GENETICS

KCNC3: phenotype, mutations, channel biophysics-a study of 260 familial ataxia patients

(2009) Karla P. Figueroa et al.   HUMAN MUTATION

Phosphorylation of ATXN1 at Ser776 in the cerebellum

(2009) Nathan D. Jorgensen et al.   JOURNAL OF NEUROCHEMISTRY

Posttranslational Modification of Ataxin-7 at Lysine 257 Prevents Autophagy-Mediated Turnover of an N-Terminal Caspase-7 Cleavage Fragment

(2009) S. Mookerjee et al.   JOURNAL OF NEUROSCIENCE

The ubiquitin-conjugating enzyme UbcH6 regulates the transcriptional repression activity of the SCA1 gene product ataxin-1

(2008) Soyeon Lee et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

UbcH6 interacts with and ubiquitinates the SCA1 gene product ataxin-1

(2008) Sunghoi Hong et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

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

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

Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1

(2008) Janghoo Lim et al.   NATURE

Casein kinase 2 interacts with and phosphorylates ataxin-3

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