Mechanisms of protein toxicity in neurodegenerative diseases

Opposing Effects of CREBBP Mutations Govern the Phenotype of Rubinstein-Taybi Syndrome and Adult SHH Medulloblastoma

(2018) Daniel J. Merk et al.   DEVELOPMENTAL CELL

Mitochondria and Mitochondrial Cascades in Alzheimer’s Disease

(2018) Russell H. Swerdlow   JOURNAL OF ALZHEIMERS DISEASE

Pfizer exits neuroscience

(2018) Asher Mullard   NATURE REVIEWS DRUG DISCOVERY

Trial of Solanezumab for Mild Dementia Due to Alzheimer’s Disease

(2018) Lawrence S. Honig et al.   NEW ENGLAND JOURNAL OF MEDICINE

Enteroviral Infection: The Forgotten Link to Amyotrophic Lateral Sclerosis?

(2018) Yuan Chao Xue et al.   Frontiers in Molecular Neuroscience

An unpredicted aggregation-critical region of the actin-polymerizing protein TRIOBP-1/Tara, determined by elucidation of its domain structure

(2017) Nicholas J. Bradshaw et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Aggregation of scaffolding protein DISC1 dysregulates phosphodiesterase 4 in Huntington’s disease

(2017) Motomasa Tanaka et al.   JOURNAL OF CLINICAL INVESTIGATION

The Stress Granule Transcriptome Reveals Principles of mRNA Accumulation in Stress Granules

(2017) Anthony Khong et al.   MOLECULAR CELL

Mutant TDP-43 does not impair mitochondrial bioenergetics in vitro and in vivo

(2017) Hibiki Kawamata et al.   Molecular Neurodegeneration

Understanding the roles of mutations in the amyloid precursor protein in Alzheimer disease

(2017) S Hunter et al.   MOLECULAR PSYCHIATRY

Cytosolic proteostasis through importing of misfolded proteins into mitochondria

(2017) Linhao Ruan et al.   NATURE

Enhancing mitochondrial proteostasis reduces amyloid-β proteotoxicity

(2017) Vincenzo Sorrentino et al.   NATURE

A toxic mutant huntingtin species is resistant to selective autophagy

(2017) Yuhua Fu et al.   Nature Chemical Biology

Zeroing in on neurodegenerative α-synuclein

(2017) Katie Kingwell   NATURE REVIEWS DRUG DISCOVERY

Next generation antibody drugs: pursuit of the 'high-hanging fruit'

(2017) Paul J. Carter et al.   NATURE REVIEWS DRUG DISCOVERY

Selective neuronal vulnerability in Parkinson disease

(2017) D. James Surmeier et al.   NATURE REVIEWS NEUROSCIENCE

Polyglutamine spinocerebellar ataxias — from genes to potential treatments

(2017) Henry L. Paulson et al.   NATURE REVIEWS NEUROSCIENCE

Regulation of motor proteins, axonal transport deficits and adult-onset neurodegenerative diseases

(2017) Scott T. Brady et al.   NEUROBIOLOGY OF DISEASE

Polyglutamine-Expanded Huntingtin Exacerbates Age-Related Disruption of Nuclear Integrity and Nucleocytoplasmic Transport

(2017) Fatima Gasset-Rosa et al.   NEURON

TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics

(2017) Ian R. Mackenzie et al.   NEURON

Lost in Transportation: Nucleocytoplasmic Transport Defects in ALS and Other Neurodegenerative Diseases

(2017) Hong Joo Kim et al.   NEURON

Mutant Huntingtin Disrupts the Nuclear Pore Complex

(2017) Jonathan C. Grima et al.   NEURON

Huntington’s Disease and Mitochondria

(2017) Mohammad Jodeiri Farshbaf et al.   NEUROTOXICITY RESEARCH

Pharmacological intervention of early neuropathy in neurodegenerative diseases

(2017) Min Jee Kwon et al.   PHARMACOLOGICAL RESEARCH

Toxic PRnpoly-dipeptides encoded by theC9orf72repeat expansion block nuclear import and export

(2017) Kevin Y. Shi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Golgi Outpost Synthesis Impaired by Toxic Polyglutamine Proteins Contributes to Dendritic Pathology in Neurons

(2017) Chang Geon Chung et al.   Cell Reports

Spatiotemporal Proteomic Profiling of Huntington’s Disease Inclusions Reveals Widespread Loss of Protein Function

(2017) Fabian Hosp et al.   Cell Reports

Huntingtin Inclusions Trigger Cellular Quiescence, Deactivate Apoptosis, and Lead to Delayed Necrosis

(2017) Yasmin M. Ramdzan et al.   Cell Reports

Neuropathological Staging of Brain Pathology in Sporadic Parkinson’s disease: Separating the Wheat from the Chaff

(2017) Heiko Braak et al.   Journal of Parkinsons Disease

Granulostasis: Protein Quality Control of RNP Granules

(2017) Simon Alberti et al.   Frontiers in Molecular Neuroscience

The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD

(2017) Brian D. Freibaum et al.   Frontiers in Molecular Neuroscience

α-synuclein transfer through tunneling nanotubes occurs in SH-SY5Y cells and primary brain pericytes from Parkinson’s disease patients

(2017) Birger Victor Dieriks et al.   Scientific Reports

α-Synuclein-carrying extracellular vesicles in Parkinson’s disease: deadly transmitters

(2016) Dimitry A. Chistiakov et al.   ACTA NEUROLOGICA BELGICA

Human-to-mouse prion-like propagation of mutant huntingtin protein

(2016) Iksoo Jeon et al.   ACTA NEUROPATHOLOGICA

Inside out: the role of nucleocytoplasmic transport in ALS and FTLD

(2016) Steven Boeynaems et al.   ACTA NEUROPATHOLOGICA

α-Synuclein in Extracellular Vesicles: Functional Implications and Diagnostic Opportunities

(2016) Camilla Lööv et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Tunneling nanotubes spread fibrillar α‐synuclein by intercellular trafficking of lysosomes

(2016) Saïda Abounit et al.   EMBO JOURNAL

Axonal transport defects are a common phenotype inDrosophilamodels of ALS

(2016) Katie R. Baldwin et al.   HUMAN MOLECULAR GENETICS

Exosomes and nanotubes: Control of immune cell communication

(2016) Kessler McCoy-Simandle et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Extracellular Vesicles Isolated from the Brains of rTg4510 Mice Seed Tau Protein Aggregation in a Threshold-dependent Manner

(2016) Juan Carlos Polanco et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

G3BP–Caprin1–USP10 complexes mediate stress granule condensation and associate with 40S subunits

(2016) Nancy Kedersha et al.   JOURNAL OF CELL BIOLOGY

Widespread transneuronal propagation of α-synucleinopathy triggered in olfactory bulb mimics prodromal Parkinson’s disease

(2016) Nolwen L. Rey et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Soluble Oligomers of PolyQ-Expanded Huntingtin Target a Multiplicity of Key Cellular Factors

(2016) Yujin E. Kim et al.   MOLECULAR CELL

Epigenetic Changes in Neurodegenerative Diseases

(2016) Min Jee Kwon et al.   MOLECULES AND CELLS

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

The antibody aducanumab reduces Aβ plaques in Alzheimer’s disease

(2016) Jeff Sevigny et al.   NATURE

The inhibition of TDP-43 mitochondrial localization blocks its neuronal toxicity

(2016) Wenzhang Wang et al.   NATURE MEDICINE

Poly(GR) in C9ORF72 -Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons

(2016) Rodrigo Lopez-Gonzalez et al.   NEURON

Mutations associated with familial Parkinson’s disease alter the initiation and amplification steps of α-synuclein aggregation

(2016) Patrick Flagmeier et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

α-Synuclein binds to TOM20 and inhibits mitochondrial protein import in Parkinson’s disease

(2016) Roberto Di Maio et al.   Science Translational Medicine

Principles and Properties of Stress Granules

(2016) David S.W. Protter et al.   TRENDS IN CELL BIOLOGY

Ubiqutination via K27 and K29 chains signals aggregation and neuronal protection of LRRK2 by WSB1

(2016) Frederick C. Nucifora et al.   Nature Communications

Molecular Mechanisms of Amyloid Oligomers Toxicity

(2016) Rakez Kayed et al.   JOURNAL OF ALZHEIMERS DISEASE

Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD

(2016) Steven Boeynaems et al.   Scientific Reports

Tau pathology spread in PS19 tau transgenic mice following locus coeruleus (LC) injections of synthetic tau fibrils is determined by the LC’s afferent and efferent connections

(2015) Michiyo Iba et al.   ACTA NEUROPATHOLOGICA

Induction of α-synuclein aggregate formation by CSF exosomes from patients with Parkinson’s disease and dementia with Lewy bodies

(2015) Anne Stuendl et al.   BRAIN

Brain propagation of transduced α-synuclein involves non-fibrillar protein species and is enhanced in α-synuclein null mice

(2015) Michael Helwig et al.   BRAIN

Progression of Seed-Induced Aβ Deposition within the Limbic Connectome

(2015) Lan Ye et al.   BRAIN PATHOLOGY

5′ UTR m6A Promotes Cap-Independent Translation

(2015) Kate D. Meyer et al.   CELL

Identification of Genetic Factors that Modify Clinical Onset of Huntington’s Disease

(2015) Jong-Min Lee et al.   CELL

α-Synuclein Shows High Affinity Interaction with Voltage-dependent Anion Channel, Suggesting Mechanisms of Mitochondrial Regulation and Toxicity in Parkinson Disease

(2015) Tatiana K. Rostovtseva et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport

(2015) Ke Zhang et al.   NATURE

α-Synuclein strains cause distinct synucleinopathies after local and systemic administration

(2015) W. Peelaerts et al.   NATURE

GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport

(2015) Brian D. Freibaum et al.   NATURE

Depletion of microglia and inhibition of exosome synthesis halt tau propagation

(2015) Hirohide Asai et al.   NATURE NEUROSCIENCE

From Intrinsic Firing Properties to Selective Neuronal Vulnerability in Neurodegenerative Diseases

(2015) Francesco Roselli et al.   NEURON

Transcellular spreading of huntingtin aggregates in theDrosophilabrain

(2015) Daniel T. Babcock et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Differential effects of Ydj1 and Sis1 on Hsp70-mediated clearance of stress granules inSaccharomyces cerevisiae

(2015) Robert W. Walters et al.   RNA

Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA

(2015) A. C. Woerner et al.   SCIENCE

Alzheimer's and Parkinson's diseases: The prion concept in relation to assembled A , tau, and  -synuclein

(2015) M. Goedert   SCIENCE

Human endogenous retrovirus-K contributes to motor neuron disease

(2015) Wenxue Li et al.   Science Translational Medicine

Prion-like transmission of neuronal huntingtin aggregates to phagocytic glia in the Drosophila brain

(2015) Margaret M. P. Pearce et al.   Nature Communications

Depressive symptoms in neurodegenerative diseases

(2015) Miquel Baquero   World Journal of Clinical Cases

Is there a risk of prion-like disease transmission by Alzheimer- or Parkinson-associated protein particles?

(2014) Michael Beekes et al.   ACTA NEUROPATHOLOGICA

Direct evidence of Parkinson pathology spread from the gastrointestinal tract to the brain in rats

(2014) Staffan Holmqvist et al.   ACTA NEUROPATHOLOGICA

Liquid-Liquid Phase Separation in Biology

(2014) Anthony A. Hyman et al.   Annual Review of Cell and Developmental Biology

A Mitocentric View of Parkinson's Disease

(2014) Nele A. Haelterman et al.   Annual Review of Neuroscience

A huntingtin-mediated fast stress response halting endosomal trafficking is defective in Huntington's disease

(2014) Siddharth Nath et al.   HUMAN MOLECULAR GENETICS

Multiple Factors Contribute to the Peripheral Induction of Cerebral  -Amyloidosis

(2014) Y. S. Eisele et al.   JOURNAL OF NEUROSCIENCE

Profilin 1 Associates with Stress Granules and ALS-Linked Mutations Alter Stress Granule Dynamics

(2014) M. D. Figley et al.   JOURNAL OF NEUROSCIENCE

Inhibition of mitochondrial protein import by mutant huntingtin

(2014) Hiroko Yano et al.   NATURE NEUROSCIENCE

Exosome reduction in vivo is associated with lower amyloid plaque load in the 5XFAD mouse model of Alzheimer's disease

(2014) Michael B. Dinkins et al.   NEUROBIOLOGY OF AGING

Aggregation of the Protein TRIOBP-1 and Its Potential Relevance to Schizophrenia

(2014) Nicholas J. Bradshaw et al.   PLoS One

Intramuscular injection of  -synuclein induces CNS  -synuclein pathology and a rapid-onset motor phenotype in transgenic mice

(2014) A. N. Sacino et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Differential ubiquitination and degradation of huntingtin fragments modulated by ubiquitin-protein ligase E3A

(2014) K. P. Bhat et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Aggregation of polyglutamine-expanded ataxin-3 sequesters its specific interacting partners into inclusions: Implication in a loss-of-function pathology

(2014) Hui Yang et al.   Scientific Reports

Alzheimer’s disease drug-development pipeline: few candidates, frequent failures

(2014) Jeffrey L Cummings et al.   Alzheimers Research & Therapy

Long-Term Health of Dopaminergic Neuron Transplants in Parkinson's Disease Patients

(2014) Penelope J. Hallett et al.   Cell Reports

Ubiquitin pathways in neurodegenerative disease

(2014) Graham Atkin et al.   Frontiers in Molecular Neuroscience

The ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solution

(2014) Nico P. Dantuma et al.   Frontiers in Molecular Neuroscience

Neuron-to-neuron wild-type Tau protein transfer through a trans-synaptic mechanism: relevance to sporadic tauopathies

(2014) Simon Dujardin et al.   Acta Neuropathologica Communications

Pathological alpha-synuclein propagates through neural networks

(2014) Masami Masuda-Suzukake et al.   Acta Neuropathologica Communications

Peripheral administration of tau aggregates triggers intracerebral tauopathy in transgenic mice

(2013) Florence Clavaguera et al.   ACTA NEUROPATHOLOGICA

Therapeutic induction of autophagy to modulate neurodegenerative disease progression

(2013) Warren E Hochfeld et al.   ACTA PHARMACOLOGICA SINICA

A novel method for detecting 7-methyl guanine reveals aberrant methylation levels in Huntington disease

(2013) Beena Thomas et al.   ANALYTICAL BIOCHEMISTRY

Prion-like spreading of pathological α-synuclein in brain

(2013) Masami Masuda-Suzukake et al.   BRAIN

Eukaryotic Stress Granules Are Cleared by Autophagy and Cdc48/VCP Function

(2013) J. Ross Buchan et al.   CELL

Dual Specificity Kinase DYRK3 Couples Stress Granule Condensation/Dissolution to mTORC1 Signaling

(2013) Frank Wippich et al.   CELL

Motor neuron disease and frontotemporal dementia: sometimes related, sometimes not

(2013) John Hardy et al.   EXPERIMENTAL NEUROLOGY

A Rapid Gene Delivery-Based Mouse Model for Early-Stage Alzheimer Disease-Type Tauopathy

(2013) Robert Siman et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Amyotrophic lateral sclerosis-linked FUS/TLS alters stress granule assembly and dynamics

(2013) Desiree M Baron et al.   Molecular Neurodegeneration

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

(2013) Hong Joo Kim et al.   NATURE

Self-propagation of pathogenic protein aggregates in neurodegenerative diseases

(2013) Mathias Jucker et al.   NATURE

The role of autophagy in neurodegenerative disease

(2013) Ralph A Nixon   NATURE MEDICINE

Interplay of LRRK2 with chaperone-mediated autophagy

(2013) Samantha J Orenstein et al.   NATURE NEUROSCIENCE

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

(2013) Boxun Lu et al.   NATURE NEUROSCIENCE

Choosing an animal model for the study of Huntington's disease

(2013) Mahmoud A. Pouladi et al.   NATURE REVIEWS NEUROSCIENCE

Transglutaminase and Polyamination of Tubulin: Posttranslational Modification for Stabilizing Axonal Microtubules

(2013) Yuyu Song et al.   NEURON

Extensive changes in DNA methylation are associated with expression of mutant huntingtin

(2013) C. W. Ng et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Molecular chaperone Hsp110 rescues a vesicle transport defect produced by an ALS-associated mutant SOD1 protein in squid axoplasm

(2013) Y. Song et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The C9orf72 GGGGCC Repeat Is Translated into Aggregating Dipeptide-Repeat Proteins in FTLD/ALS

(2013) K. Mori et al.   SCIENCE

CHIP has a protective role against oxidative stress-induced cell death through specific regulation of Endonuclease G

(2013) J S Lee et al.   Cell Death & Disease

A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases

(2013) Kyota Fujita et al.   Nature Communications

Soluble forms of polyQ-expanded huntingtin rather than large aggregates cause endoplasmic reticulum stress

(2013) Julia Leitman et al.   Nature Communications

Physiological Phenotype and Vulnerability in Parkinson's Disease

(2013) D. J. Surmeier et al.   Cold Spring Harbor Perspectives in Medicine

Genetics of Parkinson's Disease

(2013) C. Klein et al.   Cold Spring Harbor Perspectives in Medicine

Evaluation of Potential Infectivity of Alzheimer and Parkinson Disease Proteins in Recipients of Cadaver-Derived Human Growth Hormone

(2013) David J. Irwin et al.   JAMA Neurology

The Synaptic Accumulation of Hyperphosphorylated Tau Oligomers in Alzheimer Disease Is Associated With Dysfunction of the Ubiquitin-Proteasome System

(2012) Hwan-Ching Tai et al.   AMERICAN JOURNAL OF PATHOLOGY

Huntingtin protein interactions altered by polyglutamine expansion as determined by quantitative proteomic analysis

(2012) Tamara Ratovitski et al.   CELL CYCLE

Axonal degeneration in Alzheimer's disease: When signaling abnormalities meet the axonal transport system

(2012) Nicholas M. Kanaan et al.   EXPERIMENTAL NEUROLOGY

Proteomic, genomic and translational approaches identify CRMP1 for a role in schizophrenia and its underlying traits

(2012) Verian Bader et al.   HUMAN MOLECULAR GENETICS

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

Proteomic Analysis of Wild-type and Mutant Huntingtin-associated Proteins in Mouse Brains Identifies Unique Interactions and Involvement in Protein Synthesis

(2012) Brady P. Culver et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Intracerebral inoculation of pathological α-synuclein initiates a rapidly progressive neurodegenerative α-synucleinopathy in mice

(2012) Kelvin C. Luk et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Transient Sequestration of TORC1 into Stress Granules during Heat Stress

(2012) Terunao Takahara et al.   MOLECULAR CELL

Trans-Synaptic Spread of Tau Pathology In Vivo

(2012) Li Liu et al.   PLoS One

Alpha-Synuclein Cell-to-Cell Transfer and Seeding in Grafted Dopaminergic Neurons In Vivo

(2012) Elodie Angot et al.   PLoS One

Human P301L-Mutant Tau Expression in Mouse Entorhinal-Hippocampal Network Causes Tau Aggregation and Presynaptic Pathology but No Cognitive Deficits

(2012) Julie A. Harris et al.   PLoS One

The Role of Chaperone-Mediated Autophagy in Huntingtin Degradation

(2012) Lin Qi et al.   PLoS One

Aggregated proteins in schizophrenia and other chronic mental diseases

(2012) Carsten Korth   Prion

Pathological  -Synuclein Transmission Initiates Parkinson-like Neurodegeneration in Nontransgenic Mice

(2012) K. C. Luk et al.   SCIENCE

The presence of Aβ seeds, and not age per se, is critical to the initiation of Aβ deposition in the brain

(2011) Tsuyoshi Hamaguchi et al.   ACTA NEUROPATHOLOGICA

Biophysics of α-synuclein membrane interactions

(2011) Candace M. Pfefferkorn et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES

Hallucinations in Neurodegenerative Diseases

(2011) Lothar Burghaus et al.   CNS Neuroscience & Therapeutics

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

(2011) Pelagia Deriziotis et al.   EMBO JOURNAL

TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1

(2011) Karli K. McDonald et al.   HUMAN MOLECULAR GENETICS

Exosome-associated Tau Is Secreted in Tauopathy Models and Is Selectively Phosphorylated in Cerebrospinal Fluid in Early Alzheimer Disease

(2011) Sudad Saman et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

α-Synuclein propagates from mouse brain to grafted dopaminergic neurons and seeds aggregation in cultured human cells

(2011) Christian Hansen et al.   JOURNAL OF CLINICAL INVESTIGATION

Regulation of the Human Papillomavirus Type 18 E6/E6AP Ubiquitin Ligase Complex by the HECT Domain-Containing Protein EDD

(2011) V. Tomaic et al.   JOURNAL OF VIROLOGY

Transfer of host-derived alpha synuclein to grafted dopaminergic neurons in rat

(2011) Jeffrey H. Kordower et al.   NEUROBIOLOGY OF DISEASE

Selective Neuronal Vulnerability in Neurodegenerative Diseases: from Stressor Thresholds to Degeneration

(2011) Smita Saxena et al.   NEURON

Alpha-synuclein release by neurons activates the inflammatory response in a microglial cell line

(2011) Lydia Alvarez-Erviti et al.   NEUROSCIENCE RESEARCH

Alcohol Exposure Decreases CREB Binding Protein Expression and Histone Acetylation in the Developing Cerebellum

(2011) Weixiang Guo et al.   PLoS One

Pathogenic polyglutamine proteins cause dendrite defects associated with specific actin cytoskeletal alterations in Drosophila

(2011) S. B. Lee et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Exercise and Genetic Rescue of SCA1 via the Transcriptional Repressor Capicua

(2011) J. D. Fryer et al.   SCIENCE

ADF/Cofilin-Actin Rods in Neurodegenerative Diseases

(2010) J.R. Bamburg et al.   Current Alzheimer Research

Autophagy and misfolded proteins in neurodegeneration

(2010) Daniel J. Metcalf et al.   EXPERIMENTAL NEUROLOGY

Review Article: Genetics of Alzheimer Disease

(2010) Lynn M. Bekris et al.   JOURNAL OF GERIATRIC PSYCHIATRY AND NEUROLOGY

Cell-Produced  -Synuclein Is Secreted in a Calcium-Dependent Manner by Exosomes and Impacts Neuronal Survival

(2010) E. Emmanouilidou et al.   JOURNAL OF NEUROSCIENCE

TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia

(2010) Ian RA Mackenzie et al.   LANCET NEUROLOGY

Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease

(2010) Marta Martinez-Vicente et al.   NATURE NEUROSCIENCE

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

(2010) Daryl A Bosco et al.   NATURE NEUROSCIENCE

Chaperone-mediated autophagy dysfunction in the pathogenesis of neurodegeneration

(2010) Hiroshi Koga et al.   NEUROBIOLOGY OF DISEASE

Transsynaptic Progression of Amyloid-β-Induced Neuronal Dysfunction within the Entorhinal-Hippocampal Network

(2010) Julie A. Harris et al.   NEURON

Deficits in axonal transport precede ALS symptoms in vivo

(2010) L. G. Bilsland et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Peripherally Applied A -Containing Inoculates Induce Cerebral  -Amyloidosis

(2010) Y. S. Eisele et al.   SCIENCE

Autophagy, a guardian against neurodegeneration

(2010) Moisés García-Arencibia et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Molecular Neuropathology of TDP-43 Proteinopathies

(2009) Manuela Neumann   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Axonal Transport Defects in Neurodegenerative Diseases

(2009) G. A. Morfini et al.   JOURNAL OF NEUROSCIENCE

Transmission and spreading of tauopathy in transgenic mouse brain

(2009) Florence Clavaguera et al.   NATURE CELL BIOLOGY

Inclusion formation and neuronal cell death through neuron-to-neuron transmission of  -synuclein

(2009) P. Desplats 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

Cytoskeletal pathologies of Alzheimer disease

(2009) James R. Bamburg et al.   CELL MOTILITY AND THE CYTOSKELETON

Nuclear accumulation of polyglutamine disease proteins and neuropathology

(2009) Lauren S Havel et al.   Molecular Brain

Nuclear bodies in neurodegenerative disease

(2008) John Woulfe   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Homozygosity mapping in a family presenting with schizophrenia, epilepsy and hearing impairment

(2008) Helen M Knight et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

Impaired ERAD and ER stress are early and specific events in polyglutamine toxicity

(2008) M. L. Duennwald et al.   GENES & DEVELOPMENT

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

Impaired Protein Aggregate Handling and Clearance Underlie the Pathogenesis of p97/VCP-associated Disease

(2008) Jeong-Sun Ju et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

Dopamine neurons implanted into people with Parkinson's disease survive without pathology for 14 years

(2008) Ivar Mendez et al.   NATURE MEDICINE

Lewy bodies in grafted neurons in subjects with Parkinson's disease suggest host-to-graft disease propagation

(2008) Jia-Yi Li et al.   NATURE MEDICINE

Cyclophilin D deficiency attenuates mitochondrial and neuronal perturbation and ameliorates learning and memory in Alzheimer's disease

(2008) Heng Du et al.   NATURE MEDICINE

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