A fine balance between Prpf19 and Exoc7 in achieving degradation of aggregated protein and suppression of cell death in spinocerebellar ataxia type 3

Isolation and Identification of a Novel Anti-protein Aggregation Activity of Lignin-Carbohydrate Complex From Chionanthus retusus Leaves

(2020) Wenhui Pei et al.   Frontiers in Bioengineering and Biotechnology

FipoQ/FBXO33, a Cullin-1-based ubiquitin ligase complex component modulates ubiquitination and solubility of polyglutamine disease protein

(2019) Zhefan Stephen Chen et al.   JOURNAL OF NEUROCHEMISTRY

A Peptidylic Inhibitor for Neutralizing (GGGGCC)-Associated Neurodegeneration in C9ALS-FTD

(2019) Qian Zhang et al.   Molecular Therapy-Nucleic Acids

Druggable genome screen identifies new regulators of the abundance and toxicity of ATXN3, the Spinocerebellar Ataxia type 3 disease protein

(2019) Naila S. Ashraf et al.   NEUROBIOLOGY OF DISEASE

Pathogenesis of SCA3 and implications for other polyglutamine diseases

(2019) Hayley S. McLoughlin et al.   NEUROBIOLOGY OF DISEASE

PolyQ-expanded huntingtin and ataxin-3 sequester ubiquitin adaptors hHR23B and UBQLN2 into aggregates via conjugated ubiquitin

(2018) Hui Yang et al.   FASEB JOURNAL

Machado-Joseph disease / Spinocerebellar ataxia type 3:lessons from disease pathogenesis and clues into therapy

(2018) Carlos Matos et al.   JOURNAL OF NEUROCHEMISTRY

Transcriptional Dysregulation and Post-translational Modifications in Polyglutamine Diseases: From Pathogenesis to Potential Therapeutic Strategies

(2018) Chunchen Xiang et al.   Frontiers in Molecular Neuroscience

Polyglutamine Repeats in Neurodegenerative Diseases

(2018) Andrew P. Lieberman et al.   Annual Review of Pathology-Mechanisms of Disease

Drosophila Exo70 Is Essential for Neurite Extension and Survival under Thermal Stress

(2018) Alex Chun Koon et al.   JOURNAL OF NEUROSCIENCE

AQAMAN, a bisamidine-based inhibitor of toxic protein inclusions in neurons, ameliorates cytotoxicity in polyglutamine disease models

(2018) Huiling Hong et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

An essential domain of an early-diverged RNA polymerase II functions to accurately decode a primitive chromatin landscape

(2017) Anish Das et al.   NUCLEIC ACIDS RESEARCH

Treatment with Caffeic Acid and Resveratrol Alleviates Oxidative Stress Induced Neurotoxicity in Cell and Drosophila Models of Spinocerebellar Ataxia Type3

(2017) Yu-Ling Wu et al.   Scientific Reports

On the distribution of intranuclear and cytoplasmic aggregates in the brainstem of patients with spinocerebellar ataxia type 2 and 3

(2016) Kay Seidel et al.   BRAIN PATHOLOGY

Pancreatic cancer risk variant in LINC00673 creates a miR-1231 binding site and interferes with PTPN11 degradation

(2016) Jian Zheng et al.   NATURE GENETICS

Structural insights into the catalysis and regulation of E3 ubiquitin ligases

(2016) Lori Buetow et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Mahogunin ring finger 1 suppresses misfolded polyglutamine aggregation and cytotoxicity

(2014) Deepak Chhangani et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Ubiquitin ligase ITCH recruitment suppresses the aggregation and cellular toxicity of cytoplasmic misfolded proteins

(2014) Deepak Chhangani et al.   Scientific Reports

The U-box-type ubiquitin ligase PRP19β regulates astrocyte differentiation via ubiquitination of PTP1B

(2013) Takeyuki Yamada et al.   BRAIN RESEARCH

PolyQ Proteins Interfere with Nuclear Degradation of Cytosolic Proteins by Sequestering the Sis1p Chaperone

(2013) Sae-Hun Park et al.   CELL

Exo70 Generates Membrane Curvature for Morphogenesis and Cell Migration

(2013) Yuting Zhao et al.   DEVELOPMENTAL CELL

Ubiquitination Regulates the Neuroprotective Function of the Deubiquitinase Ataxin-3in Vivo

(2013) Wei-Ling Tsou et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Exo70E2 is essential for exocyst subunit recruitment and EXPO formation in both plants and animals

(2013) Yu Ding et al.   MOLECULAR BIOLOGY OF THE CELL

H1152 promotes the degradation of polyglutamine-expanded ataxin-3 or ataxin-7 independently of its ROCK-inhibiting effect and ameliorates mutant ataxin-3-induced neurodegeneration in the SCA3 transgenic mouse

(2013) Hung-Li Wang et al.   NEUROPHARMACOLOGY

Degradation of mutant huntingtin via the ubiquitin/proteasome system is modulated by FE65

(2012) Wan Ning Vanessa Chow et al.   BIOCHEMICAL JOURNAL

Exo70 Subunit of the Exocyst Complex Is Involved in Adhesion-Dependent Trafficking of Caveolin-1

(2012) Maud Hertzog et al.   PLoS One

CAG expansion induces nucleolar stress in polyglutamine diseases

(2012) H. Tsoi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Exo70, a subunit of the exocyst complex, interacts with SNEVhPrp19/hPso4and is involved in pre-mRNA splicing

(2011) Hanna Dellago et al.   BIOCHEMICAL JOURNAL

Expanded polyglutamine domain possesses nuclear export activity which modulates subcellular localization and toxicity of polyQ disease protein via exportin-1

(2011) Wing Man Chan et al.   HUMAN MOLECULAR GENETICS

The Prp19 complex and the Usp4Sart3 deubiquitinating enzyme control reversible ubiquitination at the spliceosome

(2010) E. J. Song et al.   GENES & DEVELOPMENT

A mitochondrial ubiquitin ligase MITOL controls cell toxicity of polyglutamine-expanded protein

(2010) Ayumu Sugiura et al.   MITOCHONDRION

Enhancement of proteasome activity by a small-molecule inhibitor of USP14

(2010) Byung-Hoon Lee et al.   NATURE

Gp78, an ER associated E3, promotes SOD1 and ataxin-3 degradation

(2009) Zheng Ying et al.   HUMAN MOLECULAR GENETICS

Nuclear accumulation of polyglutamine disease proteins and neuropathology

(2009) Lauren S Havel et al.   Molecular Brain

Sodium dodecyl sulfate-insoluble oligomers are involved in polyglutamine degeneration

(2008) S. L. Alan Wong et al.   FASEB JOURNAL

E6-AP Promotes Misfolded Polyglutamine Proteins for Proteasomal Degradation and Suppresses Polyglutamine Protein Aggregation and Toxicity

(2008) Amit Mishra et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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