The Ubiquitin-Proteasome System in Neurodegeneration

Enhancement of brain-type creatine kinase activity ameliorates neuronal deficits in Huntington's disease

(2013) Yow-Sien Lin et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Ubiquilin-2 (UBQLN2) binds with high affinity to the C-terminal region of TDP-43 and modulates TDP-43 levels in H4 cells: Characterization of inhibition by nucleic acids and 4-aminoquinolines

(2013) Joel A. Cassel et al.   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

Neuronal Ubiquitin Homeostasis

(2013) Jada Hallengren et al.   CELL BIOCHEMISTRY AND BIOPHYSICS

Ubiquitinated Proteins Activate the Proteasomal ATPases by Binding to Usp14 or Uch37 Homologs

(2013) Andreas Peth et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The role of autophagy in neurodegenerative disease

(2013) Ralph A Nixon   NATURE MEDICINE

VCP Is Essential for Mitochondrial Quality Control by PINK1/Parkin and this Function Is Impaired by VCP Mutations

(2013) Nam Chul Kim et al.   NEURON

The Ubiquitin-Proteasome System and the Autophagic-Lysosomal System in Alzheimer Disease

(2013) Y. Ihara et al.   Cold Spring Harbor Perspectives in Medicine

The genetics and neuropathology of Parkinson’s disease

(2012) Henry Houlden et al.   ACTA NEUROPATHOLOGICA

The genetics and neuropathology of Alzheimer’s disease

(2012) Gerard D. Schellenberg et al.   ACTA NEUROPATHOLOGICA

Impaired proteasome function in sporadic amyotrophic lateral sclerosis

(2012) Edor Kabashi et al.   Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis: New Insights into Underlying Molecular Mechanisms and Opportunities for Therapeutic Intervention

(2012) Mauro Cozzolino et al.   ANTIOXIDANTS & REDOX SIGNALING

Genetic Determinants of Amyotrophic Lateral Sclerosis as Therapeutic Targets

(2012) Daryl A. Bosco et al.   CNS & Neurological Disorders-Drug Targets

Phenotypic variability in three families withvalosin-containing proteinmutation

(2012) S. Spina et al.   EUROPEAN JOURNAL OF NEUROLOGY

Loss of HDAC6, a novel CHIP substrate, alleviates abnormal tau accumulation

(2012) Casey Cook et al.   HUMAN MOLECULAR GENETICS

Dysfunction of the Ubiquitin Ligase Ube3a May Be Associated with Synaptic Pathophysiology in a Mouse Model of Huntington Disease

(2012) Megha Maheshwari et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Motor Neuron-specific Disruption of Proteasomes, but Not Autophagy, Replicates Amyotrophic Lateral Sclerosis

(2012) Yoshitaka Tashiro et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Indirect inhibition of 26S proteasome activity in a cellular model of Huntington’s disease

(2012) Mark S. Hipp et al.   JOURNAL OF CELL BIOLOGY

Failure of Amino Acid Homeostasis Causes Cell Death following Proteasome Inhibition

(2012) Amila Suraweera et al.   MOLECULAR CELL

RPN-6 determines C. elegans longevity under proteotoxic stress conditions

(2012) David Vilchez et al.   NATURE

Valosin-containing protein (VCP) mutations in sporadic amyotrophic lateral sclerosis

(2012) Yevgeniya Abramzon et al.   NEUROBIOLOGY OF AGING

UBQLN2 mutations are rare in French and French–Canadian amyotrophic lateral sclerosis

(2012) Hussein Daoud et al.   NEUROBIOLOGY OF AGING

E6-AP association promotes SOD1 aggresomes degradation and suppresses toxicity

(2012) Amit Mishra et al.   NEUROBIOLOGY OF AGING

Activity-Dependent Growth of New Dendritic Spines Is Regulated by the Proteasome

(2012) Andrew M. Hamilton et al.   NEURON

Adeno-associated virus (AAV) gene therapy for neurological disease

(2012) Marc S. Weinberg et al.   NEUROPHARMACOLOGY

A Novel “Molecular Tweezer” Inhibitor of α-Synuclein Neurotoxicity in Vitro and in Vivo

(2012) Shubhangi Prabhudesai et al.   Neurotherapeutics

Ubiquilin-1 regulates amyloid precursor protein maturation and degradation by stimulating K63-linked polyubiquitination of lysine 688

(2012) A. El Ayadi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Decreased Proteasomal Activity Causes Age-Related Phenotypes and Promotes the Development of Metabolic Abnormalities

(2011) Utano Tomaru et al.   AMERICAN JOURNAL OF PATHOLOGY

Altered neurotransmitter release machinery in mice deficient for the deubiquitinating enzyme Usp14

(2011) Bula J. Bhattacharyya et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Amyloid-like Aggregates Sequester Numerous Metastable Proteins with Essential Cellular Functions

(2011) Heidi Olzscha et al.   CELL

Structural plasticity of dendritic spines

(2011) Miquel Bosch et al.   CURRENT OPINION IN NEUROBIOLOGY

Misfolded PrP impairs the UPS by interaction with the 20S proteasome and inhibition of substrate entry

(2011) Pelagia Deriziotis et al.   EMBO JOURNAL

Selective neuroprotective effects of the S18Y polymorphic variant of UCH-L1 in the dopaminergic system

(2011) Maria Xilouri et al.   HUMAN MOLECULAR GENETICS

Broad activation of the ubiquitin–proteasome system by Parkin is critical for mitophagy

(2011) Nickie C. Chan et al.   HUMAN MOLECULAR GENETICS

Ubiquilin-1 Is a Molecular Chaperone for the Amyloid Precursor Protein

(2011) Emily S. Stieren et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Parkin Mediates Proteasome-dependent Protein Degradation and Rupture of the Outer Mitochondrial Membrane

(2011) Saori R. Yoshii et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Ubiquitin Homeostasis Is Critical for Synaptic Development and Function

(2011) P.-C. Chen et al.   JOURNAL OF NEUROSCIENCE

Distinct Roles In Vivo for the Ubiquitin-Proteasome System and the Autophagy-Lysosomal Pathway in the Degradation of  -Synuclein

(2011) D. Ebrahimi-Fakhari et al.   JOURNAL OF NEUROSCIENCE

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

(2011) Han-Xiang Deng et al.   NATURE

Molecular chaperones in protein folding and proteostasis

(2011) F. Ulrich Hartl et al.   NATURE

Protein standard absolute quantification (PSAQ) method for the measurement of cellular ubiquitin pools

(2011) Stephen E Kaiser et al.   NATURE METHODS

Expression of Mutant or Cytosolic PrP in Transgenic Mice and Cells Is Not Associated with Endoplasmic Reticulum Stress or Proteasome Dysfunction

(2011) Elena Quaglio et al.   PLoS One

Adeno-Associated Virus-Mediated Rescue of the Cognitive Defects in a Mouse Model for Angelman Syndrome

(2011) Jennifer L. Daily et al.   PLoS One

The proteasomal subunit Rpn6 is a molecular clamp holding the core and regulatory subcomplexes together

(2011) G. R. Pathare et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

 -Synuclein fate is determined by USP9X-regulated monoubiquitination

(2011) R. Rott 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

Proteostasis and Movement Disorders: Parkinson's Disease and Amyotrophic Lateral Sclerosis

(2011) D. A. Bosco et al.   Cold Spring Harbor Perspectives in Biology

Oxidative protein damage and the proteasome

(2010) S. Grimm et al.   AMINO ACIDS

Tau protein degradation is catalyzed by the ATP/ubiquitin-independent 20S proteasome under normal cell conditions

(2010) Tilman Grune et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD

(2010) Emilie Tresse et al.   Autophagy

The Angelman Syndrome Protein Ube3A Regulates Synapse Development by Ubiquitinating Arc

(2010) Paul L. Greer et al.   CELL

Autophosphorylated CaMKIIα Acts as a Scaffold to Recruit Proteasomes to Dendritic Spines

(2010) Baris Bingol et al.   CELL

The small heat shock protein B8 (HspB8) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS)

(2010) Valeria Crippa et al.   HUMAN MOLECULAR GENETICS

Proteasome and p97 mediate mitophagy and degradation of mitofusins induced by Parkin

(2010) Atsushi Tanaka et al.   JOURNAL OF CELL BIOLOGY

Acute Polyglutamine Expression in Inducible Mouse Model Unravels Ubiquitin/Proteasome System Impairment and Permanent Recovery Attributable to Aggregate Formation

(2010) Z. Ortega et al.   JOURNAL OF NEUROSCIENCE

Parkin Directly Modulates 26S Proteasome Activity

(2010) J. W. Um et al.   JOURNAL OF NEUROSCIENCE

Huntington's disease: from molecular pathogenesis to clinical treatment

(2010) Christopher A Ross et al.   LANCET NEUROLOGY

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

(2010) Byung-Hoon Lee et al.   NATURE

Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS

(2010) Daryl A Bosco et al.   NATURE NEUROSCIENCE

Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS

(2010) Janel O. Johnson et al.   NEURON

Mechanisms of rotenone-induced proteasome inhibition

(2010) Arthur P. Chou et al.   NEUROTOXICOLOGY

Degradation of Postsynaptic Scaffold GKAP and Regulation of Dendritic Spine Morphology by the TRIM3 Ubiquitin Ligase in Rat Hippocampal Neurons

(2010) Albert Y. Hung et al.   PLoS One

Novel Antibodies Reveal Inclusions Containing Non-Native SOD1 in Sporadic ALS Patients

(2010) Karin Forsberg et al.   PLoS One

Assembly, structure, and function of the 26S proteasome

(2010) Lynn Bedford et al.   TRENDS IN CELL BIOLOGY

Mitophagy: the latest problem for Parkinson's disease

(2010) Cristofol Vives-Bauza et al.   TRENDS IN MOLECULAR MEDICINE

Mitochondrial Function, Morphology, and Axonal Transport in Amyotrophic Lateral Sclerosis

(2009) Jordi Magrané et al.   ANTIOXIDANTS & REDOX SIGNALING

Oxidized Mitochondrial Protein Degradation and Repair in Aging and Oxidative Stress

(2009) Nicolas Ugarte et al.   ANTIOXIDANTS & REDOX SIGNALING

Isolation of Mammalian 26S Proteasomes and p97/VCP Complexes Using the Ubiquitin-like Domain from HHR23B Reveals Novel Proteasome-Associated Proteins†

(2009) Henrike C. Besche et al.   BIOCHEMISTRY

Mechanisms of cross-talk between the ubiquitin-proteasome and autophagy-lysosome systems

(2009) Viktor I. Korolchuk et al.   FEBS LETTERS

Parkin promotes intracellular A 1-42 clearance

(2009) M. P. Burns et al.   HUMAN MOLECULAR GENETICS

VCPMutations Causing Frontotemporal Lobar Degeneration Disrupt Localization of TDP-43 and Induce Cell Death

(2009) Michael A. Gitcho et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Regulation of the Proteasome by Neuronal Activity and Calcium/Calmodulin-dependent Protein Kinase II

(2009) Stevan N. Djakovic et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease

(2009) Jeong-Sun Ju et al.   JOURNAL OF CELL BIOLOGY

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

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

Polymorphism of HD and UCHL-1 genes in Huntington’s disease

(2009) Er-he Xu et al.   JOURNAL OF CLINICAL NEUROSCIENCE

The ubiquitin ligase E6-AP promotes degradation of α-synuclein

(2009) Shalaka A. Mulherkar et al.   JOURNAL OF NEUROCHEMISTRY

The Proteasome-Associated Deubiquitinating Enzyme Usp14 Is Essential for the Maintenance of Synaptic Ubiquitin Levels and the Development of Neuromuscular Junctions

(2009) P.-C. Chen et al.   JOURNAL OF NEUROSCIENCE

Parkin reverses intracellular β-amyloid accumulation and its negative effects on proteasome function

(2009) Kenneth M. Rosen et al.   JOURNAL OF NEUROSCIENCE RESEARCH

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

(2009) Andreas Peth et al.   MOLECULAR CELL

Reciprocal Effects of α-Synuclein Overexpression and Proteasome Inhibition in Neuronal Cells and Tissue

(2009) Melanie Dyllick-Brenzinger et al.   NEUROTOXICITY RESEARCH

Parkin gene therapy

(2009) Hideki Mochizuki   PARKINSONISM & RELATED DISORDERS

Accumulation of ubiquitin conjugates in a polyglutamine disease model occurs without global ubiquitin/proteasome system impairment

(2009) C. J. Maynard et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

CHIP regulates leucine-rich repeat kinase-2 ubiquitination, degradation, and toxicity

(2009) Han Seok Ko et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Targeted intracellular protein degradation induced by a small molecule: En route to chemical proteomics

(2008) Ashley R. Schneekloth et al.   BIOORGANIC & MEDICINAL CHEMISTRY LETTERS

Protein degradation, as with protein synthesis, is required during not only long-term spatial memory consolidation but also reconsolidation

(2008) Julien Artinian et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Functional alterations of the ubiquitin-proteasome system in motor neurons of a mouse model of familial amyotrophic lateral sclerosis†

(2008) Cristina Cheroni et al.   HUMAN MOLECULAR GENETICS

Aberrant molecular properties shared by familial Parkinson’s disease-associated mutant UCH-L1 and carbonyl-modified UCH-L1

(2008) Tomohiro Kabuta et al.   HUMAN MOLECULAR GENETICS

The Deubiquitinating Enzyme Ataxin-3, a Polyglutamine Disease Protein, Edits Lys63Linkages in Mixed Linkage Ubiquitin Chains

(2008) Brett J. Winborn et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

α-Synuclein Protofibrils Inhibit 26 S Proteasome-mediated Protein Degradation

(2008) Nan-Yan Zhang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Aberrant Interaction between Parkinson Disease-associated Mutant UCH-L1 and the Lysosomal Receptor for Chaperone-mediated Autophagy

(2008) Tomohiro Kabuta et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Regulation of Postsynaptic RapGAP SPAR by Polo-like Kinase 2 and the SCFβ-TRCPUbiquitin Ligase in Hippocampal Neurons

(2008) Xiaolu L. Ang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Wild Type α-Synuclein Is Degraded by Chaperone-mediated Autophagy and Macroautophagy in Neuronal Cells

(2008) Tereza Vogiatzi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

CHIP Targets Toxic α-Synuclein Oligomers for Degradation

(2008) Julie E. Tetzlaff et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

PKA rapidly enhances proteasome assembly and activity in in vivo canine hearts

(2008) Mitsutoshi Asai et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

Proteasomes remain intact, but show early focal alteration in their composition in a mouse model of amyotrophic lateral sclerosis

(2008) Edor Kabashi et al.   JOURNAL OF NEUROCHEMISTRY

Effects of UCH-L1 on α-synuclein over-expression mouse model of Parkinson’s disease

(2008) Toru Yasuda et al.   JOURNAL OF NEUROCHEMISTRY

Blocking A 42 Accumulation Delays the Onset and Progression of Tau Pathology via the C Terminus of Heat Shock Protein70-Interacting Protein: A Mechanistic Link between A  and Tau Pathology

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

N-Terminal Mutant Huntingtin Associates with Mitochondria and Impairs Mitochondrial Trafficking

(2008) A. L. Orr et al.   JOURNAL OF NEUROSCIENCE

Depletion of 26S Proteasomes in Mouse Brain Neurons Causes Neurodegeneration and Lewy-Like Inclusions Resembling Human Pale Bodies

(2008) L. Bedford et al.   JOURNAL OF NEUROSCIENCE

Genetic Evidence Linking Age-Dependent Attenuation of the 26S Proteasome with the Aging Process

(2008) A. Tonoki et al.   MOLECULAR AND CELLULAR BIOLOGY

Long-term proteasome dysfunction in the mouse brain by expression of aberrant ubiquitin

(2008) David F. Fischer et al.   NEUROBIOLOGY OF AGING

Cell-produced α-synuclein oligomers are targeted to, and impair, the 26S proteasome

(2008) Evangelia Emmanouilidou et al.   NEUROBIOLOGY OF AGING

In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis

(2008) Aislinn J. Williams et al.   NEUROBIOLOGY OF DISEASE

Critical Role of CDK5 and Polo-like Kinase 2 in Homeostatic Synaptic Plasticity during Elevated Activity

(2008) Daniel P. Seeburg et al.   NEURON

Targeting steroid hormone receptors for ubiquitination and degradation in breast and prostate cancer

(2008) A Rodriguez-Gonzalez et al.   ONCOGENE

Hypothalamic neurodegeneration and adult-onset obesity in mice lacking the Ubb polyubiquitin gene

(2008) K.-Y. Ryu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Synaptic Protein Degradation Underlies Destabilization of Retrieved Fear Memory

(2008) S.-H. Lee et al.   SCIENCE

Aβ inhibits the proteasome and enhances amyloid and tau accumulation

(2007) Bertrand P. Tseng et al.   NEUROBIOLOGY OF AGING