scyllo-Inositol Promotes Robust Mutant Huntingtin Protein Degradation

Does bafilomycin A1block the fusion of autophagosomes with lysosomes?

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Combination therapy in a transgenic model of Alzheimer's disease

(2013) Nurgul Aytan et al.   EXPERIMENTAL NEUROLOGY

Prefoldin Protects Neuronal Cells from Polyglutamine Toxicity by Preventing Aggregation Formation

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The DNAJB6 and DNAJB8 Protein Chaperones Prevent Intracellular Aggregation of Polyglutamine Peptides

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Binding Mechanism of Inositol Stereoisomers to Monomers and Aggregates of Aβ(16-22)

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Identification of NUB1 as a suppressor of mutant Huntingtin toxicity via enhanced protein clearance

(2013) Boxun Lu et al.   NATURE NEUROSCIENCE

Exogenous delivery of chaperonin subunit fragment ApiCCT1 modulates mutant Huntingtin cellular phenotypes

(2013) E. M. Sontag et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Inhibition of amyloid-beta peptide aggregation rescues the autophagic deficits in the TgCRND8 mouse model of Alzheimer disease

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Integrated protein quality-control pathways regulate free  -globin in murine  -thalassemia

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Cathepsins L and Z Are Critical in Degrading Polyglutamine-containing Proteins within Lysosomes

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Sustained Therapeutic Reversal of Huntington's Disease by Transient Repression of Huntingtin Synthesis

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The Role of Chaperone-Mediated Autophagy in Huntingtin Degradation

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Protein aggregates in Huntington's disease

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An aggregation sensing reporter identifies leflunomide and teriflunomide as polyglutamine aggregate inhibitors

(2011) Rodrigo A. Fuentealba et al.   HUMAN MOLECULAR GENETICS

Soluble Aβ oligomer production and toxicity

(2011) Megan E. Larson et al.   JOURNAL OF NEUROCHEMISTRY

Autophagy deregulation in neurodegenerative diseases - recent advances and future perspectives

(2011) Zelda H. Cheung et al.   JOURNAL OF NEUROCHEMISTRY

Binding of Inositol Stereoisomers To Model Amyloidogenic Peptides

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Reduction of mutant huntingtin accumulation and toxicity by lysosomal cathepsins D and B in neurons

(2011) Qiuli Liang et al.   Molecular Neurodegeneration

Identifying polyglutamine protein species in situ that best predict neurodegeneration

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Sodium/myo-Inositol Transporters: Substrate Transport Requirements and Regional Brain Expression in the TgCRND8 Mouse Model of Amyloid Pathology

(2011) Daniela Fenili et al.   PLoS One

Bifunctional Anti-Huntingtin Proteasome-Directed Intrabodies Mediate Efficient Degradation of Mutant Huntingtin Exon 1 Protein Fragments

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The Parkinson's disease protein LRRK2 impairs proteasome substrate clearance without affecting proteasome catalytic activity

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Lysosomal Proteolysis and Autophagy Require Presenilin 1 and Are Disrupted by Alzheimer-Related PS1 Mutations

(2010) Ju-Hyun Lee et al.   CELL

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

Quantitative Relationships between Huntingtin Levels, Polyglutamine Length, Inclusion Body Formation, and Neuronal Death Provide Novel Insight into Huntington's Disease Molecular Pathogenesis

(2010) J. Miller et al.   JOURNAL OF NEUROSCIENCE

Huntington's disease: from molecular pathogenesis to clinical treatment

(2010) Christopher A Ross et al.   LANCET NEUROLOGY

Prion-like transmission of protein aggregates in neurodegenerative diseases

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Methylene blue fails to inhibit Tau and polyglutamine protein dependent toxicity in zebrafish

(2010) Frauke van Bebber et al.   NEUROBIOLOGY OF DISEASE

Inducible over-expression of wild type α-synuclein in human neuronal cells leads to caspase-dependent non-apoptotic death

(2009) Kostas Vekrellis et al.   JOURNAL OF NEUROCHEMISTRY

Intrabody Gene Therapy Ameliorates Motor, Cognitive, and Neuropathological Symptoms in Multiple Mouse Models of Huntington's Disease

(2009) A. L. Southwell et al.   JOURNAL OF NEUROSCIENCE

Ubiquitin Chain Editing Revealed by Polyubiquitin Linkage-Specific Antibodies

(2008) Kim Newton et al.   CELL

Huntington’s disease: degradation of mutant huntingtin by autophagy

(2008) Sovan Sarkar et al.   FEBS Journal

Single Neuron Ubiquitin-Proteasome Dynamics Accompanying Inclusion Body Formation in Huntington Disease

(2008) Siddhartha Mitra 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

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

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