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

Arginine Methylation: The Coming of Age

(2017) Roméo S. Blanc et al.   MOLECULAR CELL

Pathology of Neurodegenerative Diseases

(2017) Brittany N. Dugger et al.   Cold Spring Harbor Perspectives in Biology

Glycolytic-to-oxidative fiber-type switch and mTOR signaling activation are early-onset features of SBMA muscle modified by high-fat diet

(2016) Anna Rocchi et al.   ACTA NEUROPATHOLOGICA

The phosphatase calcineurin regulates pathological TDP-43 phosphorylation

(2016) Nicole F. Liachko et al.   ACTA NEUROPATHOLOGICA

Monomethylated and unmethylated FUS exhibit increased binding to Transportin and distinguish FTLD-FUS from ALS-FUS

(2016) Marc Suárez-Calvet et al.   ACTA NEUROPATHOLOGICA

Acetylation within the First 17 Residues of Huntingtin Exon 1 Alters Aggregation and Lipid Binding

(2016) Maxmore Chaibva et al.   BIOPHYSICAL JOURNAL

Pathogenic Mutations in the Valosin-containing Protein/p97(VCP) N-domain Inhibit the SUMOylation of VCP and Lead to Impaired Stress Response

(2016) Tao Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

Serine 421 regulates mutant huntingtin toxicity and clearance in mice

(2016) Ian H. Kratter et al.   JOURNAL OF CLINICAL INVESTIGATION

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

Adenylyl cyclase activating polypeptide reduces phosphorylation and toxicity of the polyglutamine-expanded androgen receptor in spinobulbar muscular atrophy

(2016) M. J. Polanco et al.   Science Translational Medicine

A Phosphomimetic Mutation Stabilizes SOD1 and Rescues Cell Viability in the Context of an ALS-Associated Mutation

(2016) James M. Fay et al.   STRUCTURE

Blocking the association of HDAC4 with MAP1S accelerates autophagy clearance of mutant Huntingtin

(2015) Fei Yue et al.   Aging-US

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

PRMT5- mediated symmetric arginine dimethylation is attenuated by mutant huntingtin and is impaired in Huntington's disease (HD)

(2015) Tamara Ratovitski et al.   CELL CYCLE

Serine phosphorylation and arginine methylation at the crossroads to neurodegeneration

(2015) Manuela Basso et al.   EXPERIMENTAL NEUROLOGY

Disrupting SUMOylation enhances transcriptional function and ameliorates polyglutamine androgen receptor–mediated disease

(2015) Jason P. Chua et al.   JOURNAL OF CLINICAL INVESTIGATION

Treatment with a Global Methyltransferase Inhibitor Induces the Intranuclear Aggregation of ALS-Linked FUS Mutant In Vitro

(2015) Sakiko Fujii et al.   NEUROCHEMICAL RESEARCH

Inhibition of Pathogenic Mutant SOD1 Aggregation in Cultured Motor Neuronal Cells by Prevention of Its SUMOylation on Lysine 75

(2015) Audrey Dangoumau et al.   Neurodegenerative Diseases

Protein Arginine Methyltransferase 6 Enhances Polyglutamine-Expanded Androgen Receptor Function and Toxicity in Spinal and Bulbar Muscular Atrophy

(2015) Chiara Scaramuzzino et al.   NEURON

Defective Proteasome Delivery of Polyubiquitinated Proteins by Ubiquilin-2 Proteins Containing ALS Mutations

(2015) Lydia Chang et al.   PLoS One

An acetylation switch controls TDP-43 function and aggregation propensity

(2015) Todd J. Cohen et al.   Nature Communications

DYRK2 Negatively Regulates Type I Interferon Induction by Promoting TBK1 Degradation via Ser527 Phosphorylation

(2015) Tai An et al.   PLoS Pathogens

Cytoplasmic sequestration of FUS/TLS associated with ALS alters histone marks through loss of nuclear protein arginine methyltransferase 1

(2014) Michael Tibshirani et al.   HUMAN MOLECULAR GENETICS

UBE2E Ubiquitin-conjugating Enzymes and Ubiquitin Isopeptidase Y Regulate TDP-43 Protein Ubiquitination

(2014) Friederike Hans et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

TDP-43 modification in the hSOD1G93Aamyotrophic lateral sclerosis mouse model

(2014) MuDan Cai et al.   NEUROLOGICAL RESEARCH

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

(2014) Akiko Asada et al.   NEUROSCIENCE LETTERS

SUMO3 Modification Accelerates the Aggregation of ALS-Linked SOD1 Mutants

(2014) Takako Niikura et al.   PLoS One

The Tau Tubulin Kinases TTBK1/2 Promote Accumulation of Pathological TDP-43

(2014) Nicole F. Liachko et al.   PLoS Genetics

CDC7 inhibition blocks pathological TDP-43 phosphorylation and neurodegeneration

(2013) Nicole F. Liachko et al.   ANNALS OF NEUROLOGY

Sumoylation: A Regulatory Protein Modification in Health and Disease

(2013) Annette Flotho et al.   Annual Review of Biochemistry

TDP-43 Phosphorylation by casein kinase Iε promotes oligomerization and enhances toxicity in vivo

(2013) Darshana K. Choksi et al.   HUMAN MOLECULAR GENETICS

Parkin reverses TDP-43-induced cell death and failure of amino acid homeostasis

(2013) Michaeline Hebron et al.   JOURNAL OF NEUROCHEMISTRY

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(2013) Carlo Rinaldi et al.   MOLECULAR MEDICINE

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

Protein Arginine Methyltransferase 1 and 8 Interact with FUS to Modify Its Sub-Cellular Distribution and Toxicity In Vitro and In Vivo

(2013) Chiara Scaramuzzino et al.   PLoS One

Arginine methylation next to the PY-NLS modulates Transportin binding and nuclear import of FUS

(2012) Dorothee Dormann et al.   EMBO JOURNAL

Selective histone deacetylase (HDAC) inhibition imparts beneficial effects in Huntington's disease mice: implications for the ubiquitin–proteasomal and autophagy systems

(2012) Haiqun Jia et al.   HUMAN MOLECULAR GENETICS

Parkin Ubiquitinates Tar-DNA Binding Protein-43 (TDP-43) and Promotes Its Cytosolic Accumulation via Interaction with Histone Deacetylase 6 (HDAC6)

(2012) Michaeline L. Hebron et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Arginine methylation by PRMT1 regulates nuclear-cytoplasmic localization and toxicity of FUS/TLS harbouring ALS-linked mutations

(2011) Miranda L. Tradewell et al.   HUMAN MOLECULAR GENETICS

SIRT1 Modulates Aggregation and Toxicity through Deacetylation of the Androgen Receptor in Cell Models of SBMA

(2011) H. L. Montie et al.   JOURNAL OF NEUROSCIENCE

Hyperphosphorylation as a Defense Mechanism to Reduce TDP-43 Aggregation

(2011) Huei-Ying Li et al.   PLoS One

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

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

TLS and PRMT1 synergistically coactivate transcription at the survivin promoter through TLS arginine methylation

(2010) Kun Du et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Regulation of TDP-43 aggregation by phosphorylation andp62/SQSTM1

(2010) Owen A. Brady et al.   JOURNAL OF NEUROCHEMISTRY

Phosphorylation Promotes Neurotoxicity in a Caenorhabditis elegans Model of TDP-43 Proteinopathy

(2010) N. F. Liachko et al.   JOURNAL OF NEUROSCIENCE

B2 attenuates polyglutamine-expanded androgen receptor toxicity in cell and fly models of spinal and bulbar muscular atrophy

(2010) Isabella Palazzolo et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Multiplex SILAC Analysis of a Cellular TDP-43 Proteinopathy Model Reveals Protein Inclusions Associated with SUMOylation and Diverse Polyubiquitin Chains

(2010) Nicholas T. Seyfried et al.   MOLECULAR & CELLULAR PROTEOMICS

Phosphorylation regulates proteasomal-mediated degradation and solubility of TAR DNA binding protein-43 C-terminal fragments

(2010) Yong-Jie Zhang et al.   Molecular Neurodegeneration

Native Functions of the Androgen Receptor Are Essential to Pathogenesis in a Drosophila Model of Spinobulbar Muscular Atrophy

(2010) Natalia B. Nedelsky et al.   NEURON

Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies

(2009) Manuela Neumann et al.   ACTA NEUROPATHOLOGICA

Acetylation Targets Mutant Huntingtin to Autophagosomes for Degradation

(2009) Hyunkyung Jeong et al.   CELL

Histone Deacetylase Inhibitors and Neurodegenerative Disorders: Holding the Promise

(2009) Antonello Mai et al.   CURRENT PHARMACEUTICAL DESIGN

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

Phosphorylation of Threonine 3

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

Small Ubiquitin-like Modifier (SUMO) Modification of the Androgen Receptor Attenuates Polyglutamine-mediated Aggregation

(2009) Sarmistha Mukherjee et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome

(2009) Leslie Michels Thompson et al.   JOURNAL OF CELL BIOLOGY

Phosphorylation of ATXN1 at Ser776 in the cerebellum

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

Proteolytic processing of TAR DNA binding protein-43 by caspases produces C-terminal fragments with disease defining properties independent of progranulin

(2009) Dorothee Dormann 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

Overexpression of IGF-1 in Muscle Attenuates Disease in a Mouse Model of Spinal and Bulbar Muscular Atrophy

(2009) Isabella Palazzolo et al.   NEURON

Serines 13 and 16 Are Critical Determinants of Full-Length Human Mutant Huntingtin Induced Disease Pathogenesis in HD Mice

(2009) Xiaofeng Gu et al.   NEURON

Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity

(2009) Y.-J. Zhang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

(2008) Masato Hasegawa et al.   ANNALS OF NEUROLOGY

PRMT1 mediated methylation of TAF15 is required for its positive gene regulatory function

(2008) Laure Jobert et al.   EXPERIMENTAL CELL RESEARCH

Abnormal phosphorylation of Ser409/410 of TDP-43 in FTLD-U and ALS

(2008) Yuki Inukai et al.   FEBS LETTERS

Potentiation of Amyotrophic Lateral Sclerosis (ALS)-associated TDP-43 Aggregation by the Proteasome-targeting Factor, Ubiquilin 1

(2008) Sang Hwa Kim et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Phosphorylation of Profilin by ROCK1 Regulates Polyglutamine Aggregation

(2008) J. Shao et al.   MOLECULAR AND CELLULAR BIOLOGY

Casein kinase 2 interacts with and phosphorylates ataxin-3

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