The Ubiquitination, Disaggregation and Proteasomal Degradation Machineries in Polyglutamine Disease

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 E3 ubiquitin ligase CHIP selectively regulates mutant epidermal growth factor receptor by ubiquitination and degradation

(2016) Chaeuk Chung et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Atypical ubiquitination by E3 ligase WWP1 inhibits the proteasome-mediated degradation of mutant huntingtin

(2016) Li Lin et al.   BRAIN RESEARCH

UBQLN2 Mediates Autophagy-Independent Protein Aggregate Clearance by the Proteasome

(2016) Roland Hjerpe et al.   CELL

Compartment-Dependent Degradation of Mutant Huntingtin Accounts for Its Preferential Accumulation in Neuronal Processes

(2016) T. Zhao et al.   JOURNAL OF NEUROSCIENCE

Discovery of Therapeutic Approaches for Polyglutamine Diseases: A Summary of Recent Efforts

(2016) Sofia Esteves et al.   MEDICINAL RESEARCH REVIEWS

Modulation of Molecular Chaperones in Huntington’s Disease and Other Polyglutamine Disorders

(2016) Sara D. Reis et al.   MOLECULAR NEUROBIOLOGY

Rescue of Metabolic Alterations in AR113Q Skeletal Muscle by Peripheral Androgen Receptor Gene Silencing

(2016) Elisa Giorgetti et al.   Cell Reports

Cytoplasmic Ubiquitin-Specific Protease 19 (USP19) Modulates Aggregation of Polyglutamine-Expanded Ataxin-3 and Huntingtin through the HSP90 Chaperone

(2016) Wen-Tian He et al.   PLoS One

Targeting Hsp90/Hsp70-Based Protein Quality Control for Treatment of Adult Onset Neurodegenerative Diseases

(2015) William B. Pratt et al.   Annual Review of Pharmacology and Toxicology

Silencing neuronal mutant androgen receptor in a mouse model of spinal and bulbar muscular atrophy

(2015) Kentaro Sahashi et al.   HUMAN MOLECULAR GENETICS

Aggregation of Polyglutamine-expanded Ataxin 7 Protein Specifically Sequesters Ubiquitin-specific Protease 22 and Deteriorates Its Deubiquitinating Function in the Spt-Ada-Gcn5-Acetyltransferase (SAGA) Complex

(2015) Hui Yang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Deubiquitinating Enzyme USP7 Regulates Androgen Receptor Activity by Modulating Its Binding to Chromatin

(2015) Shu-Ting Chen et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Role of the Protein Quality Control System in SBMA

(2015) Paola Rusmini et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

Autophagy in Huntington disease and huntingtin in autophagy

(2015) Dale D.O. Martin et al.   TRENDS IN NEUROSCIENCES

Aberrant Autophagic Response in The Muscle of A Knock-in Mouse Model of Spinal and Bulbar Muscular Atrophy

(2015) Paola Rusmini et al.   Scientific Reports

A model in which heat shock protein 90 targets protein-folding clefts: Rationale for a new approach to neuroprotective treatment of protein folding diseases

(2014) William B Pratt et al.   EXPERIMENTAL BIOLOGY AND MEDICINE

Ubiquitin-specific protease-14 reduces cellular aggregates and protects against mutant huntingtin-induced cell degeneration: involvement of the proteasome and ER stress-activated kinase IRE1 

(2014) A. Hyrskyluoto et al.   HUMAN MOLECULAR GENETICS

Peripheral Androgen Receptor Gene Suppression Rescues Disease in Mouse Models of Spinal and Bulbar Muscular Atrophy

(2014) Andrew P. Lieberman et al.   Cell Reports

USP22 Regulates Oncogenic Signaling Pathways to Drive Lethal Cancer Progression

(2013) R. S. Schrecengost et al.   CANCER RESEARCH

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

(2013) Sae-Hun Park et al.   CELL

Deubiquitinating Enzyme Usp12 Is a Novel Co-activator of the Androgen Receptor

(2013) Urszula L. Burska et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

(2013) Yuting Shi et al.   PLoS One

Activation of Hsp70 reduces neurotoxicity by promoting polyglutamine protein degradation

(2012) Adrienne M Wang et al.   Nature Chemical Biology

Impaired motoneuronal retrograde transport in two models of SBMA implicates two sites of androgen action

(2011) Michael Q. Kemp et al.   HUMAN MOLECULAR GENETICS

Tumor Necrosis Factor Receptor-associated Factor 6 (TRAF6) Associates with Huntingtin Protein and Promotes Its Atypical Ubiquitination to Enhance Aggregate Formation

(2011) Silvia Zucchelli et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Ordered Assembly of Heat Shock Proteins, Hsp26, Hsp70, Hsp90, and Hsp104, on Expanded Polyglutamine Fragments Revealed by Chemical Probes

(2011) Gladis M. Walter et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Ube2w and Ataxin-3 Coordinately Regulate the Ubiquitin Ligase CHIP

(2011) K. Matthew Scaglione et al.   MOLECULAR CELL

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

USP10 deubiquitylates the histone variant H2A.Z and both are required for androgen receptor-mediated gene activation

(2011) Ryan Draker et al.   NUCLEIC ACIDS RESEARCH

Inhibiting the ubiquitin–proteasome system leads to preferential accumulation of toxic N-terminal mutant huntingtin fragments

(2010) Xiang Li et al.   HUMAN MOLECULAR GENETICS

Inhibition of Hsp70 by Methylene Blue Affects Signaling Protein Function and Ubiquitination and Modulates Polyglutamine Protein Degradation

(2010) Adrienne M. Wang 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

The Deubiquitinating Enzyme USP26 Is a Regulator of Androgen Receptor Signaling

(2010) A. M. G. Dirac et al.   MOLECULAR CANCER RESEARCH

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

(2010) Byung-Hoon Lee et al.   NATURE

Dynamic Cycling with Hsp90 Stabilizes Neuronal Nitric Oxide Synthase Through Calmodulin-Dependent Inhibition of Ubiquitination

(2009) Hwei-Ming Peng et al.   BIOCHEMISTRY

Ubiquitinated Proteins Activate the Proteasome by Binding to Usp14/Ubp6, which Causes 20S Gate Opening

(2009) Andreas Peth et al.   MOLECULAR CELL

The Ubiquitin-Proteasome Reporter GFPu Does Not Accumulate in Neurons of the R6/2 Transgenic Mouse Model of Huntington's Disease

(2009) John S. Bett et al.   PLoS One

17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in an SBMA model mouse

(2008) Keisuke Tokui et al.   HUMAN MOLECULAR GENETICS

CHIP deletion reveals functional redundancy of E3 ligases in promoting degradation of both signaling proteins and expanded glutamine proteins

(2008) Y. Morishima et al.   HUMAN MOLECULAR GENETICS

Mitochondrial abnormalities in spinal and bulbar muscular atrophy

(2008) Srikanth Ranganathan et al.   HUMAN MOLECULAR GENETICS

Impaired ubiquitin–proteasome system activity in the synapses of Huntington's disease mice

(2008) Jianjun Wang et al.   JOURNAL OF CELL BIOLOGY

Differential Activities of the Ubiquitin-Proteasome System in Neurons versus Glia May Account for the Preferential Accumulation of Misfolded Proteins in Neurons

(2008) S. Tydlacka et al.   JOURNAL OF NEUROSCIENCE