All Roads Lead to Rome: Different Molecular Players Converge to Common Toxic Pathways in Neurodegeneration

Metabolic and immune dysfunction of glia in neurodegenerative disorders: Focus on iPSC models

(2021) Taisia Rõlova et al.   STEM CELLS

Tau Post-translational Modifications: Dynamic Transformers of Tau Function, Degradation, and Aggregation

(2021) Carolina Alquezar et al.   Frontiers in Neurology

Chemical chaperones targeted to the endoplasmic reticulum (ER) and lysosome prevented neurodegeneration in a C9orf72 repeat expansion drosophila amyotrophic lateral sclerosis (ALS) model

(2021) Salome Azoulay-Ginsburg et al.   Pharmacological Reports

Skeletal Muscle in ALS: An Unappreciated Therapeutic Opportunity?

(2021) Silvia Scaricamazza et al.   Cells

A promising new therapy for ALS

(2021) Heather Wood   Nature Reviews Neurology

Role of a Heat Shock Transcription Factor and the Major Heat Shock Protein Hsp70 in Memory Formation and Neuroprotection

(2021) Olga G. Zatsepina et al.   Cells

The Mitochondrial Hsp90 TRAP1 and Alzheimer’s Disease

(2021) Françoise A. Dekker et al.   Frontiers in Molecular Biosciences

Interaction of α-synuclein and Parkin in iron toxicity on SH-SY5Y cells: implications in the pathogenesis of Parkinson's disease

(2020) Upasana Ganguly et al.   BIOCHEMICAL JOURNAL

The contribution of Parkin, PINK1 and DJ‐1 genes to selective neuronal degeneration in Parkinson's disease

(2020) Marc van der Vlag et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Mutations in Superoxide Dismutase 1 (Sod1) Linked to Familial Amyotrophic Lateral Sclerosis Can Disrupt High-Affinity Zinc-Binding Promoted by the Copper Chaperone for Sod1 (Ccs)

(2020) Stefanie D. Boyd et al.   MOLECULES

Ambroxol for the Treatment of Patients With Parkinson Disease With and Without Glucocerebrosidase Gene Mutations

(2020) Stephen Mullin et al.   JAMA Neurology

The involvement of stress granules in aging and aging‐associated diseases

(2020) Xiuling Cao et al.   AGING CELL

Liquid–Liquid Phase Separation and Its Mechanistic Role in Pathological Protein Aggregation

(2020) W. Michael Babinchak et al.   JOURNAL OF MOLECULAR BIOLOGY

Parkinson’s disease treatment: past, present, and future

(2020) John D. Elsworth   JOURNAL OF NEURAL TRANSMISSION

Reduced C9ORF72 function exacerbates gain of toxicity from ALS/FTD-causing repeat expansion in C9orf72

(2020) Qiang Zhu et al.   NATURE NEUROSCIENCE

Genetics of Parkinson's disease: An introspection of its journey towards precision medicine

(2020) Sara Bandres-Ciga et al.   NEUROBIOLOGY OF DISEASE

Amyotrophic lateral sclerosis: a clinical review

(2020) P. Masrori et al.   EUROPEAN JOURNAL OF NEUROLOGY

Truncation of tau selectively facilitates its pathological activities

(2020) Jianlan Gu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Medical Management and Prevention of Motor Complications in Parkinson’s Disease

(2020) Stephen D. Aradi et al.   Neurotherapeutics

Neurotrophic factors for disease-modifying treatments of Parkinson's disease: gaps between basic science and clinical studies

(2020) Piotr Chmielarz et al.   Pharmacological Reports

Aducanumab, gantenerumab, BAN2401, and ALZ-801—the first wave of amyloid-targeting drugs for Alzheimer’s disease with potential for near term approval

(2020) Martin Tolar et al.   Alzheimers Research & Therapy

Identity by descent analysis identifies founder events and links SOD1 familial and sporadic ALS cases

(2020) Lyndal Henden et al.   npj Genomic Medicine

Molecular and pharmacological chaperones for SOD1

(2020) Gareth S.A. Wright   BIOCHEMICAL SOCIETY TRANSACTIONS

Small molecule therapeutics for tauopathy in Alzheimer's disease: Walking on the path of most resistance

(2020) Lisha Wang et al.   EUROPEAN JOURNAL OF MEDICINAL CHEMISTRY

ALS-Related Mutant SOD1 Aggregates Interfere with Mitophagy by Sequestering the Autophagy Receptor Optineurin

(2020) Yeong Jin Tak et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Amyloid‐β impairs the phagocytosis of dystrophic synapses by astrocytes in Alzheimer's disease

(2020) Maria V. Sanchez‐Mico et al.   GLIA

Skeletal Muscle-Restricted Expression of Human SOD1 in Transgenic Mice Causes a Fatal ALS-Like Syndrome

(2020) Lee J. Martin et al.   Frontiers in Neurology

Molecular Mechanisms of TDP-43 Misfolding and Pathology in Amyotrophic Lateral Sclerosis

(2019) Archana Prasad et al.   Frontiers in Molecular Neuroscience

Mitophagy inhibits amyloid-β and tau pathology and reverses cognitive deficits in models of Alzheimer’s disease

(2019) Evandro F. Fang et al.   NATURE NEUROSCIENCE

Transmission of α-synuclein seeds in neurodegenerative disease: recent developments

(2019) Richard J. Karpowicz et al.   LABORATORY INVESTIGATION

The role of liquid-liquid phase separation in aggregation of the TDP-43 low complexity domain

(2019) W. Michael Babinchak et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Deletion of Tbk1 disrupts autophagy and reproduces behavioral and locomotor symptoms of FTD-ALS in mice

(2019) Weisong Duan et al.   Aging-US

Tau immunotherapies for Alzheimer’s disease

(2019) Justin L. Hoskin et al.   EXPERT OPINION ON INVESTIGATIONAL DRUGS

Oxidative Stress: A Key Modulator in Neurodegenerative Diseases

(2019) Anju Singh et al.   MOLECULES

The role of C9orf72 in neurodegenerative disorders: a systematic review, an updated meta-analysis, and the creation of an online database

(2019) Chrysoula Marogianni et al.   NEUROBIOLOGY OF AGING

Exploring the Extended Biological Functions of the Human Copper Chaperone of Superoxide Dismutase 1

(2019) Yan Ge et al.   PROTEIN JOURNAL

Ubiquitin Signaling and Degradation of Aggregate-Prone Proteins

(2019) Margarita Galves et al.   TRENDS IN BIOCHEMICAL SCIENCES

Regional High Iron in the Substantia Nigra Differentiates Parkinson’s Disease Patients From Healthy Controls

(2019) Kiarash Ghassaban et al.   Frontiers in Aging Neuroscience

Overriding FUS autoregulation in mice triggers gain-of-toxic dysfunctions in RNA metabolism and autophagy-lysosome axis

(2019) Shuo-Chien Ling et al.   eLife

The Pathobiology of TDP-43 C-Terminal Fragments in ALS and FTLD

(2019) Britt A. Berning et al.   Frontiers in Neuroscience

Amyloid Beta and Phosphorylated Tau-Induced Defective Autophagy and Mitophagy in Alzheimer’s Disease

(2019) P. Hemachandra Reddy et al.   Cells

Epidemiology of amyotrophic lateral sclerosis

(2019) Elisa Longinetti et al.   CURRENT OPINION IN NEUROLOGY

Emerging therapies in Huntington’s disease

(2019) Hassaan Bashir   Expert Review of Neurotherapeutics

Small-Molecule Modulation of TDP-43 Recruitment to Stress Granules Prevents Persistent TDP-43 Accumulation in ALS/FTD

(2019) Mark Y. Fang et al.   NEURON

Transneuronal Propagation of Pathologic α-Synuclein from the Gut to the Brain Models Parkinson’s Disease

(2019) Sangjune Kim et al.   NEURON

Endo-lysosomal pathway and ubiquitin-proteasome system dysfunction in Alzheimer's disease pathogenesis

(2019) Jiqing Cao et al.   NEUROSCIENCE LETTERS

AAV2/9-mediated overexpression of MIF inhibits SOD1 misfolding, delays disease onset, and extends survival in mouse models of ALS

(2019) Marcel F. Leyton-Jaimes et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

SOD1-positive aggregate accumulation in the CNS predicts slower disease progression and increased longevity in a mutant SOD1 mouse model of ALS

(2019) Cindy Gill et al.   Scientific Reports

Masitinib as an add-on therapy to riluzole in patients with amyotrophic lateral sclerosis: a randomized clinical trial

(2019) Jesus S. Mora et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Protein quality control in the secretory pathway

(2019) Zhihao Sun et al.   JOURNAL OF CELL BIOLOGY

The pathobiology of perturbed mutant huntingtin protein–protein interactions in Huntington's disease

(2019) Erich E. Wanker et al.   JOURNAL OF NEUROCHEMISTRY

The neuropathological diagnosis of Alzheimer’s disease

(2019) Michael A. DeTure et al.   Molecular Neurodegeneration

Axonal transport and neurological disease

(2019) James N. Sleigh et al.   Nature Reviews Neurology

A VDAC1-Derived N-Terminal Peptide Inhibits Mutant SOD1-VDAC1 Interactions and Toxicity in the SOD1 Model of ALS

(2019) Anna Shteinfer-Kuzmine et al.   Frontiers in Cellular Neuroscience

Tau Pathology in Chronic Traumatic Encephalopathy and Alzheimer's Disease: Similarities and Differences

(2019) Atsuko Katsumoto et al.   Frontiers in Neurology

The lipophilic 4‐phenylbutyric acid derivative prevents aggregation and retention of misfolded proteins

(2019) Salome Azoulay-Ginsburg et al.   CHEMISTRY-A EUROPEAN JOURNAL

Imaging of post-mortem human brain tissue using electron and X-ray microscopy

(2019) Amanda J Lewis et al.   CURRENT OPINION IN STRUCTURAL BIOLOGY

Hsp90 and Its Co-Chaperones in Neurodegenerative Diseases

(2019) Anastasiia Bohush et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Lowering Mutant Huntingtin Levels and Toxicity: Autophagy-Endolysosome Pathways in Huntington's Disease

(2019) Evelina Valionyte et al.   JOURNAL OF MOLECULAR BIOLOGY

Exome sequencing in amyotrophic lateral sclerosis implicates a novel gene, DNAJC7, encoding a heat-shock protein

(2019) Sali M. K. Farhan et al.   NATURE NEUROSCIENCE

Modulation of actin polymerization affects nucleocytoplasmic transport in multiple forms of amyotrophic lateral sclerosis

(2019) Anthony Giampetruzzi et al.   Nature Communications

A resurrection of aducanumab for Alzheimer's disease

(2019) Lon Schneider   LANCET NEUROLOGY

Invited Review: The role of prion‐like mechanisms in neurodegenerative diseases

(2019) Z. Jaunmuktane et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Amyloid Beta and Tau Cooperate to Cause Reversible Behavioral and Transcriptional Deficits in a Model of Alzheimer’s Disease

(2019) Eleanor K. Pickett et al.   Cell Reports

The cholinergic system in the pathophysiology and treatment of Alzheimer’s disease

(2018) Harald Hampel et al.   BRAIN

Stress Granule Assembly Disrupts Nucleocytoplasmic Transport

(2018) Ke Zhang et al.   CELL

Exploring the genetics and non-cell autonomous mechanisms underlying ALS/FTLD

(2018) Hongbo Chen et al.   CELL DEATH AND DIFFERENTIATION

Tau in Alzheimer's Disease: Mechanisms and Therapeutic Strategies

(2018) Yu Gao et al.   Current Alzheimer Research

Mitochondrial Dysfunction in Parkinson’s Disease: New Mechanistic Insights and Therapeutic Perspectives

(2018) Jin-Sung Park et al.   Current Neurology and Neuroscience Reports

Macrophage migration inhibitory factor: A multifaceted cytokine implicated in multiple neurological diseases

(2018) Marcel F. Leyton-Jaimes et al.   EXPERIMENTAL NEUROLOGY

TDP-43 post-translational modifications in health and disease

(2018) Emanuele Buratti   EXPERT OPINION ON THERAPEUTIC TARGETS

Brain Aging and Late-Onset Alzheimer’s Disease: A Matter of Increased Amyloid or Reduced Energy?

(2018) Patrizia Mecocci et al.   JOURNAL OF ALZHEIMERS DISEASE

Efficacy and safety of amantadine for the treatment of l-DOPA-induced dyskinesia

(2018) Santiago Perez-Lloret et al.   JOURNAL OF NEURAL TRANSMISSION

Haploinsufficiency leads to neurodegeneration in C9ORF72 ALS/FTD human induced motor neurons

(2018) Yingxiao Shi et al.   NATURE MEDICINE

TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD

(2018) Ching-Chieh Chou et al.   NATURE NEUROSCIENCE

Randomized, double-blind, placebo-controlled trial of arimoclomol in rapidly progressiveSOD1ALS

(2018) Michael Benatar et al.   NEUROLOGY

Clearance of Amyloid Beta and Tau in Alzheimer’s Disease: from Mechanisms to Therapy

(2018) Shu-Hui Xin et al.   NEUROTOXICITY RESEARCH

MIF inhibits the formation and toxicity of misfolded SOD1 amyloid aggregates: implications for familial ALS

(2018) Neta Shvil et al.   Cell Death & Disease

A complex of C9ORF72 and p62 uses arginine methylation to eliminate stress granules by autophagy

(2018) Maneka Chitiprolu et al.   Nature Communications

TDP-43 regulation of stress granule dynamics in neurodegenerative disease-relevant cell types

(2018) Yousra Khalfallah et al.   Scientific Reports

Relevance of Phosphorylation and Truncation of Tau to the Etiopathogenesis of Alzheimer’s Disease

(2018) Yan Zhou et al.   Frontiers in Aging Neuroscience

Safety and Tolerability of Multiple Ascending Doses of PRX002/RG7935, an Anti–α-Synuclein Monoclonal Antibody, in Patients With Parkinson Disease

(2018) Joseph Jankovic et al.   JAMA Neurology

The Parkinson Pandemic—A Call to Action

(2018) E. Ray Dorsey et al.   JAMA Neurology

Long-term Phase 1/2 intraspinal stem cell transplantation outcomes in ALS

(2018) Stephen A. Goutman et al.   Annals of Clinical and Translational Neurology

TBK1 Suppresses RIPK1-Driven Apoptosis and Inflammation during Development and in Aging

(2018) Daichao Xu et al.   CELL

FUS-induced neurotoxicity in Drosophila is prevented by downregulating nucleocytoplasmic transport proteins

(2018) Jolien Steyaert et al.   HUMAN MOLECULAR GENETICS

Abnormal Hyperphosphorylation of Tau: Sites, Regulation, and Molecular Mechanism of Neurofibrillary Degeneration

(2018) Jian-Zhi Wang et al.   JOURNAL OF ALZHEIMERS DISEASE

C9ORF72 Hexanucleotide Repeat Number in Frontotemporal Lobar Degeneration: A Genotype-Phenotype Correlation Study

(2018) Luisa Benussi et al.   JOURNAL OF ALZHEIMERS DISEASE

Protein misfolding, aggregation, and conformational strains in neurodegenerative diseases

(2018) Claudio Soto et al.   NATURE NEUROSCIENCE

C9orf72-mediated ALS and FTD: multiple pathways to disease

(2018) Rubika Balendra et al.   Nature Reviews Neurology

Tau Protein Disrupts Nucleocytoplasmic Transport in Alzheimer’s Disease

(2018) Bahareh Eftekharzadeh et al.   NEURON

Endoplasmic reticulum stress leads to accumulation of wild-type SOD1 aggregates associated with sporadic amyotrophic lateral sclerosis

(2018) Danilo B. Medinas et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Sources of Reactive Oxygen Species and Its Possible Role in the Pathogenesis of Parkinson’s Disease

(2018) Minrui Weng et al.   Parkinsons Disease

Mutant superoxide dismutase aggregates from human spinal cord transmit amyotrophic lateral sclerosis

(2018) Elaheh Ekhtiari Bidhendi et al.   ACTA NEUROPATHOLOGICA

Targeting the proteostasis network in Huntington’s disease

(2018) Tânia R. Soares et al.   AGEING RESEARCH REVIEWS

Hsp90 and Hsp70 chaperones: Collaborators in protein remodeling

(2018) Olivier Genest et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Huntington's disease: a clinical review

(2017) P. McColgan et al.   EUROPEAN JOURNAL OF NEUROLOGY

Amyloid precursor protein and endosomal–lysosomal dysfunction in Alzheimer’s disease: inseparable partners in a multifactorial disease

(2017) Ralph A. Nixon   FASEB JOURNAL

TDP-43 and FUS en route from the nucleus to the cytoplasm

(2017) Helena Ederle et al.   FEBS LETTERS

Proteostasis of Huntingtin in Health and Disease

(2017) Seda Koyuncu et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Memantine for Alzheimer’s Disease: An Updated Systematic Review and Meta-analysis

(2017) Taro Kishi et al.   JOURNAL OF ALZHEIMERS DISEASE

Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice

(2017) Lindsay A. Becker et al.   NATURE

A systemic view of Alzheimer disease — insights from amyloid-β metabolism beyond the brain

(2017) Jun Wang et al.   Nature Reviews Neurology

Neurobiology of axonal transport defects in motor neuron diseases: Opportunities for translational research?

(2017) Kurt J. De Vos 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

Huntington’s Disease: Nuclear Gatekeepers Under Attack

(2017) Matthew B. Veldman et al.   NEURON

Mutant Huntingtin Disrupts the Nuclear Pore Complex

(2017) Jonathan C. Grima et al.   NEURON

The role of mitochondria in amyotrophic lateral sclerosis

(2017) Emma F. Smith et al.   NEUROSCIENCE LETTERS

Huntington’s Disease and Mitochondria

(2017) Mohammad Jodeiri Farshbaf et al.   NEUROTOXICITY RESEARCH

Distinct roles for motor neuron autophagy early and late in the SOD1G93Amouse model of ALS

(2017) Noam D. Rudnick et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Factors underlying cognitive decline in old age and Alzheimer’s disease: the role of the hippocampus

(2017) Wafa Jaroudi et al.   REVIEWS IN THE NEUROSCIENCES

Juvenile Huntington’s Disease: Diagnostic and Treatment Considerations for the Psychiatrist

(2017) Joanna Quigley   Current Psychiatry Reports

Proteinopathy, oxidative stress and mitochondrial dysfunction: cross talk in Alzheimer's disease and Parkinson's disease

(2017) Gargi Ganguly et al.   Drug Design Development and Therapy

Disease Mechanisms of C9ORF72 Repeat Expansions

(2017) Tania F. Gendron et al.   Cold Spring Harbor Perspectives in Medicine

TBK1: a new player in ALS linking autophagy and neuroinflammation

(2017) James A. Oakes et al.   Molecular Brain

The Role of the Heat Shock Protein B8 (HSPB8) in Motoneuron Diseases

(2017) Paola Rusmini et al.   Frontiers in Molecular Neuroscience

Protein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia Continuum

(2017) Hamideh Shahheydari 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

Protein misfolding in neurodegenerative diseases: implications and strategies

(2017) Patrick Sweeney et al.   Translational Neurodegeneration

ALS mutant SOD1 interacts with G3BP1 and affects stress granule dynamics

(2016) Jozsef Gal et al.   ACTA NEUROPATHOLOGICA

The role of endoplasmic reticulum stress in neurodegenerative disease

(2016) Chunchen Xiang et al.   APOPTOSIS

Disturbance of proteasomal and autophagic protein degradation pathways by amyotrophic lateral sclerosis-linked mutations in ubiquilin 2

(2016) Mayuko Osaka et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Functional Impairment in Miro Degradation and Mitophagy Is a Shared Feature in Familial and Sporadic Parkinson’s Disease

(2016) Chung-Han Hsieh et al.   Cell Stem Cell

Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss

(2016) Jelena Scekic‐Zahirovic et al.   EMBO JOURNAL

Loss of C9ORF72 impairs autophagy and synergizes with polyQ Ataxin‐2 to induce motor neuron dysfunction and cell death

(2016) Chantal Sellier et al.   EMBO JOURNAL

Protein aggregation and neurodegenerative diseases: From theory to therapy

(2016) Vijay Kumar et al.   EUROPEAN JOURNAL OF MEDICINAL CHEMISTRY

Mutant SOD1 mediated pathogenesis of Amyotrophic Lateral Sclerosis

(2016) Simran J. Kaur et al.   GENE

Gain-of-function profilin 1 mutations linked to familial amyotrophic lateral sclerosis cause seed-dependent intracellular TDP-43 aggregation

(2016) Yoshinori Tanaka et al.   HUMAN MOLECULAR GENETICS

Variation in worldwide incidence of amyotrophic lateral sclerosis: a meta-analysis

(2016) Benoît Marin et al.   INTERNATIONAL JOURNAL OF EPIDEMIOLOGY

The Glycine-Alanine Dipeptide Repeat from C9orf72 Hexanucleotide Expansions Forms Toxic Amyloids Possessing Cell-to-Cell Transmission Properties

(2016) Yu-Jen Chang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The amyloid cascade hypothesis: are we poised for success or failure?

(2016) Eric Karran et al.   JOURNAL OF NEUROCHEMISTRY

Heat shock proteins as potential targets for protective strategies in neurodegeneration

(2016) Harm H Kampinga et al.   LANCET NEUROLOGY

First-in-human assessment of PRX002, an anti-α-synuclein monoclonal antibody, in healthy volunteers

(2016) Dale B. Schenk et al.   MOVEMENT DISORDERS

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

Neurodegenerative diseases

(2016) Marie-Thérèse Heemels   NATURE

The road to restoring neural circuits for the treatment of Alzheimer's disease

(2016) Rebecca G. Canter et al.   NATURE

Ageing, neurodegeneration and brain rejuvenation

(2016) Tony Wyss-Coray   NATURE

Defects in trafficking bridge Parkinson's disease pathology and genetics

(2016) Asa Abeliovich et al.   NATURE

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

(2016) Wenzhang Wang et al.   NATURE MEDICINE

Mitochondrial dysfunction associated with glucocerebrosidase deficiency

(2016) Matthew E. Gegg et al.   NEUROBIOLOGY OF DISEASE

In vitro prion-like behaviour of TDP-43 in ALS

(2016) Phillip Smethurst et al.   NEUROBIOLOGY OF DISEASE

The Prevalence of Huntington's Disease

(2016) Michael D. Rawlins et al.   NEUROEPIDEMIOLOGY

Endogenous macrophage migration inhibitory factor reduces the accumulation and toxicity of misfolded SOD1 in a mouse model of ALS

(2016) Marcel F. Leyton-Jaimes et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mutant PFN1 causes ALS phenotypes and progressive motor neuron degeneration in mice by a gain of toxicity

(2016) Chunxing Yang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ALS Mutations Disrupt Phase Separation Mediated by α-Helical Structure in the TDP-43 Low-Complexity C-Terminal Domain

(2016) Alexander E. Conicella et al.   STRUCTURE

Principles and Properties of Stress Granules

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

Potential Pathways of Abnormal Tau and α-Synuclein Dissemination in Sporadic Alzheimer's and Parkinson's Diseases

(2016) Heiko Braak et al.   Cold Spring Harbor Perspectives in Biology

Safety and Clinical Effects of Mesenchymal Stem Cells Secreting Neurotrophic Factor Transplantation in Patients With Amyotrophic Lateral Sclerosis

(2016) Panayiota Petrou et al.   JAMA Neurology

Susceptibility of Mutant SOD1 to Form a Destabilized Monomer Predicts Cellular Aggregation and Toxicity but Not In vitro Aggregation Propensity

(2016) Luke McAlary et al.   Frontiers in Neuroscience

Quantitative analysis and clinico-pathological correlations of different dipeptide repeat protein pathologies in C9ORF72 mutation carriers

(2015) Ian R. A. Mackenzie et al.   ACTA NEUROPATHOLOGICA

Semi-automated quantification of C9orf72 expansion size reveals inverse correlation between hexanucleotide repeat number and disease duration in frontotemporal degeneration

(2015) EunRan Suh et al.   ACTA NEUROPATHOLOGICA

Direct visualization of alpha-synuclein oligomers reveals previously undetected pathology in Parkinson’s disease brain

(2015) Rosalind F. Roberts et al.   BRAIN

A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation

(2015) Avinash Patel et al.   CELL

The TUDCA trial - innovative trial designs for amyotrophic lateral sclerosis drugs?

(2015) A. C. Ludolph   EUROPEAN JOURNAL OF NEUROLOGY

Reduced protein turnover mediates functional deficits in transgenic mice expressing the 25 kDa C-terminal fragment of TDP-43

(2015) Antonella Caccamo et al.   HUMAN MOLECULAR GENETICS

Nucleolar stress and impaired stress granule formation contribute to C9orf72 RAN translation-induced cytotoxicity

(2015) Zhouteng Tao et al.   HUMAN MOLECULAR GENETICS

Prevalence of Cerebral Amyloid Pathology in Persons Without Dementia

(2015) Willemijn J. Jansen et al.   JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport

(2015) Ke Zhang et al.   NATURE

Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia

(2015) Axel Freischmidt et al.   NATURE NEUROSCIENCE

Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS

(2015) Ana Jovičić et al.   NATURE NEUROSCIENCE

Clearance systems in the brain—implications for Alzheimer disease

(2015) Jenna M. Tarasoff-Conway et al.   Nature Reviews Neurology

Novel mutations support a role for Profilin 1 in the pathogenesis of ALS

(2015) Bradley N. Smith et al.   NEUROBIOLOGY OF AGING

Edaravone leads to proteome changes indicative of neuronal cell protection in response to oxidative stress

(2015) Mohammad-Saeid Jami et al.   NEUROCHEMISTRY INTERNATIONAL

UDCA exerts beneficial effect on mitochondrial dysfunction inLRRK2G2019Scarriers and in vivo

(2015) Heather Mortiboys et al.   NEUROLOGY

Macrophage Migration Inhibitory Factor as a Chaperone Inhibiting Accumulation of Misfolded SOD1

(2015) Adrian Israelson et al.   NEURON

Differential Toxicity of Nuclear RNA Foci versus Dipeptide Repeat Proteins in a Drosophila Model of C9ORF72 FTD/ALS

(2015) Helene Tran et al.   NEURON

The Roles of PINK1, Parkin, and Mitochondrial Fidelity in Parkinson’s Disease

(2015) Alicia M. Pickrell et al.   NEURON

Molecular chaperones and neuronal proteostasis

(2015) Heather L. Smith et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Mitochondrial dysfunction and mitophagy in Parkinson's: from familial to sporadic disease

(2015) Brent J. Ryan et al.   TRENDS IN BIOCHEMICAL SCIENCES

SOD1 misplacing and mitochondrial dysfunction in amyotrophic lateral sclerosis pathogenesis

(2015) Francesco Tafuri et al.   Frontiers in Cellular Neuroscience

Parkinson-causing α-synuclein missense mutations shift native tetramers to monomers as a mechanism for disease initiation

(2015) Ulf Dettmer et al.   Nature Communications

The Role of Oxidative Stress in Neurodegenerative Diseases

(2015) Geon Ha Kim et al.   Experimental Neurobiology

Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress

(2014) Yong-Jie Zhang et al.   ACTA NEUROPATHOLOGICA

C9orf72 FTLD/ALS-associated Gly-Ala dipeptide repeat proteins cause neuronal toxicity and Unc119 sequestration

(2014) Stephanie May et al.   ACTA NEUROPATHOLOGICA

Pyroglutamylated amyloid-β is associated with hyperphosphorylated tau and severity of Alzheimer’s disease

(2014) Markus Mandler et al.   ACTA NEUROPATHOLOGICA

“Structural characterization of the minimal segment of TDP-43 competent for aggregation”

(2014) Miguel Mompeán et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

Axotrophin/MARCH7 acts as an E3 ubiquitin ligase and ubiquitinates tau protein in vitro impairing microtubule binding

(2014) Katharina Flach et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansions

(2014) Johnathan Cooper-Knock et al.   BRAIN

Ubiquitin-dependent protein degradation at the yeast endoplasmic reticulum and nuclear envelope

(2014) Dimitrios Zattas et al.   CRITICAL REVIEWS IN BIOCHEMISTRY AND MOLECULAR BIOLOGY

Safety overview of FDA-approved medications for the treatment of the motor symptoms of Parkinson’s disease

(2014) Michele A Faulkner   Expert Opinion On Drug Safety

Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species

(2014) E. L. Scotter et al.   JOURNAL OF CELL SCIENCE

 -Synuclein Is Localized to Mitochondria-Associated ER Membranes

(2014) C. Guardia-Laguarta et al.   JOURNAL OF NEUROSCIENCE

C9orf72 nucleotide repeat structures initiate molecular cascades of disease

(2014) Aaron R. Haeusler et al.   NATURE

Cell-to-cell transmission of pathogenic proteins in neurodegenerative diseases

(2014) Jing L Guo et al.   NATURE MEDICINE

The amyloid state and its association with protein misfolding diseases

(2014) Tuomas P. J. Knowles et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Reduced C9orf72 protein levels in frontal cortex of amyotrophic lateral sclerosis and frontotemporal degeneration brain with the C9ORF72 hexanucleotide repeat expansion

(2014) Adrian J. Waite et al.   NEUROBIOLOGY OF AGING

Symptom onset in autosomal dominant Alzheimer disease: A systematic review and meta-analysis

(2014) D. C. Ryman et al.   NEUROLOGY

Axonal Transport: Cargo-Specific Mechanisms of Motility and Regulation

(2014) Sandra Maday et al.   NEURON

Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms

(2014) Leslie I. Grad et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins

(2014) S. Mizielinska et al.   SCIENCE

Poly-dipeptides encoded by the C9orf72 repeats bind nucleoli, impede RNA biogenesis, and kill cells

(2014) I. Kwon et al.   SCIENCE

Heat shock proteins in neurodegenerative disorders and aging

(2014) Rehana K. Leak   Journal of Cell Communication and Signaling

Brain mitochondria from DJ-1 knockout mice show increased respiration-dependent hydrogen peroxide consumption

(2014) Pamela Lopert et al.   Redox Biology

Molecular Chaperone Functions in Protein Folding and Proteostasis

(2013) Yujin E. Kim et al.   Annual Review of Biochemistry

Oxidative stress and mitochondrial dysfunction in Alzheimer's disease

(2013) Xinglong Wang et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

The product of C9orf72, a gene strongly implicated in neurodegeneration, is structurally related to DENN Rab-GEFs

(2013) Timothy P. Levine et al.   BIOINFORMATICS

Ursocholanic acid rescues mitochondrial function in common forms of familial Parkinson’s disease

(2013) Heather Mortiboys et al.   BRAIN

SOD1 Aggregation and ALS: Role of Metallation States and Disulfide Status

(2013) Yuewei Sheng et al.   CURRENT TOPICS IN MEDICINAL CHEMISTRY

Expression of ALS-linked TDP-43 mutant in astrocytes causes non-cell-autonomous motor neuron death in rats

(2013) Jianbin Tong et al.   EMBO JOURNAL

Oxidative damage to macromolecules in human Parkinson disease and the rotenone model

(2013) Laurie H. Sanders et al.   FREE RADICAL BIOLOGY AND MEDICINE

Characterization of the repeat expansion size in C9orf72 in amyotrophic lateral sclerosis and frontotemporal dementia

(2013) O. Dols-Icardo et al.   HUMAN MOLECULAR GENETICS

Characterization of PINK1 (PTEN-induced Putative Kinase 1) Mutations Associated with Parkinson Disease in Mammalian Cells andDrosophila

(2013) Saera Song et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Selective Forelimb Impairment in Rats Expressing a Pathological TDP-43 25 kDa C-terminal Fragment to Mimic Amyotrophic Lateral Sclerosis

(2013) Robert D Dayton et al.   MOLECULAR THERAPY

Parkin Plays a Role in Sporadic Parkinson's Disease

(2013) Ted M. Dawson et al.   Neurodegenerative Diseases

Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis

(2013) Shuo-Chien Ling et al.   NEURON

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Polo-like kinase 2 regulates selective autophagic  -synuclein clearance and suppresses its toxicity in vivo

(2013) A. Oueslati et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Large  -synuclein oligomers inhibit neuronal SNARE-mediated vesicle docking

(2013) B.-K. Choi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Releasing the brake: restoring fast axonal transport in neurodegenerative disorders

(2013) Maria-Victoria Hinckelmann et al.   TRENDS IN CELL BIOLOGY

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Structure and Function of Parkin, PINK1, and DJ-1, the Three Musketeers of Neuroprotection

(2013) Jean-François Trempe et al.   Frontiers in Neurology

Dipeptide repeat proteins are present in the p62 positive inclusions in patients with frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72

(2013) David MA Mann et al.   Acta Neuropathologica Communications

Cell-free Formation of RNA Granules: Low Complexity Sequence Domains Form Dynamic Fibers within Hydrogels

(2012) Masato Kato et al.   CELL

Microglia and Inflammation in Alzheimers Disease

(2012) Shweta Mandrekar-Colucci et al.   CNS & Neurological Disorders-Drug Targets

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Roles of Ataxin-2 in Pathological Cascades Mediated by TAR DNA-binding Protein 43 (TDP-43) and Fused in Sarcoma (FUS)

(2012) Yoshihiro Nihei et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Requirements for Stress Granule Recruitment of Fused in Sarcoma (FUS) and TAR DNA-binding Protein of 43 kDa (TDP-43)

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Accumulation of Toxic  -Synuclein Oligomer within Endoplasmic Reticulum Occurs in  -Synucleinopathy In Vivo

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The link between the GBA gene and parkinsonism

(2012) Ellen Sidransky et al.   LANCET NEUROLOGY

Recent Advances in α-Synuclein Functions, Advanced Glycation, and Toxicity: Implications for Parkinson’s Disease

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Living on the edge with too many mouths to feed: Why dopamine neurons die

(2012) J. Paul Bolam et al.   MOVEMENT DISORDERS

Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis

(2012) Chi-Hong Wu et al.   NATURE

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(2012) Eileen H. Bigio et al.   NEUROPATHOLOGY

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C9orf72 hexanucleotide repeat associated with amyotrophic lateral sclerosis and frontotemporal dementia forms RNA G-quadruplexes

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Changes in the solubility and phosphorylation of α-synuclein over the course of Parkinson’s disease

(2011) Jinxia Zhou et al.   ACTA NEUROPATHOLOGICA

p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND/ALS

(2011) Safa Al-Sarraj et al.   ACTA NEUROPATHOLOGICA

Cognitive Decline Typical of Frontotemporal Lobar Degeneration in Transgenic Mice Expressing the 25-kDa C-Terminal Fragment of TDP-43

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(2011) Melissa K. McCoy et al.   Autophagy

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Understanding the role of TDP-43 and FUS/TLS in ALS and beyond

(2011) Sandrine Da Cruz et al.   CURRENT OPINION IN NEUROBIOLOGY

A “Two-hit” Hypothesis for Inclusion Formation by Carboxyl-terminal Fragments of TDP-43 Protein Linked to RNA Depletion and Impaired Microtubule-dependent Transport

(2011) G. Scott Pesiridis et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The role of mitochondria in neurodegenerative diseases

(2011) Massimiliano Filosto et al.   JOURNAL OF NEUROLOGY

Functional Alterations to the Nigrostriatal System in Mice Lacking All Three Members of the Synuclein Family

(2011) S. Anwar et al.   JOURNAL OF NEUROSCIENCE

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(2011) Clive Ballard et al.   LANCET

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A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study

(2011) Ilse Gijselinck et al.   LANCET NEUROLOGY

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

(2011) Han-Xiang Deng et al.   NATURE

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(2011) F. Ulrich Hartl et al.   NATURE

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD

(2011) Alan E. Renton et al.   NEURON

In vivo demonstration that  -synuclein oligomers are toxic

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

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(2011) Todd J. Cohen et al.   TRENDS IN MOLECULAR MEDICINE

Familial Alzheimers Disease Mutations in Presenilin 1 Do Not Alter Levels of the Secreted Amyloid-β Protein Precursor Generated by β-Secretase Cleavage

(2010) C. Zhang et al.   Current Alzheimer Research

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(2010) K. Iqbal et al.   Current Alzheimer Research

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(2010) Youhna M Ayala et al.   EMBO JOURNAL

Heat-shock protein 70 modulates toxic extracellular α-synuclein oligomers and rescues trans-synaptic toxicity

(2010) Karin M. Danzer et al.   FASEB JOURNAL

Neurotoxic effects of TDP-43 overexpression in C. elegans

(2010) Peter E.A. Ash et al.   HUMAN MOLECULAR GENETICS

The “Dying-Back” Phenomenon of Motor Neurons in ALS

(2010) Michal Dadon-Nachum et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

Phosphorylation at S87 Is Enhanced in Synucleinopathies, Inhibits  -Synuclein Oligomerization, and Influences Synuclein-Membrane Interactions

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Alzheimer's disease: clinical trials and drug development

(2010) Francesca Mangialasche et al.   LANCET NEUROLOGY

Huntington's disease: from molecular pathogenesis to clinical treatment

(2010) Christopher A Ross et al.   LANCET NEUROLOGY

The role of parkin in familial and sporadic Parkinson's disease

(2010) Ted M. Dawson et al.   MOVEMENT DISORDERS

Cell-to-cell transmission of non-prion protein aggregates

(2010) Seung-Jae Lee et al.   Nature Reviews Neurology

Misfolded Mutant SOD1 Directly Inhibits VDAC1 Conductance in a Mouse Model of Inherited ALS

(2010) Adrian Israelson et al.   NEURON

Acetylation of Tau Inhibits Its Degradation and Contributes to Tauopathy

(2010) Sang-Won Min et al.   NEURON

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(2010) Henry W. Querfurth et al.   NEW ENGLAND JOURNAL OF MEDICINE

A Drosophila model for TDP-43 proteinopathy

(2010) Y. Li et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration

(2010) Hans Wils et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies

(2009) Manuela Neumann et al.   ACTA NEUROPATHOLOGICA

Biophysics of Parkinsons Disease: Structure and Aggregation of α- Synuclein

(2009) Vladimir Uversky et al.   CURRENT PROTEIN & PEPTIDE SCIENCE

Expression of TDP-43 C-terminal Fragmentsin VitroRecapitulates Pathological Features of TDP-43 Proteinopathies

(2009) Lionel M. Igaz et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Non–cell autonomous toxicity in neurodegenerative disorders: ALS and beyond

(2009) Hristelina Ilieva et al.   JOURNAL OF CELL BIOLOGY

Multiple Tight Phospholipid-Binding Modes of α-Synuclein Revealed by Solution NMR Spectroscopy

(2009) Christina R. Bodner et al.   JOURNAL OF MOLECULAR BIOLOGY

Brain-Permeable Small-Molecule Inhibitors of Hsp90 Prevent  -Synuclein Oligomer Formation and Rescue  -Synuclein-Induced Toxicity

(2009) P. Putcha et al.   JOURNAL OF PHARMACOLOGY AND EXPERIMENTAL THERAPEUTICS

ER Stress and Unfolded Protein Response in Amyotrophic Lateral Sclerosis

(2009) Kohsuke Kanekura et al.   MOLECULAR NEUROBIOLOGY

Prion-like mechanisms in neurodegenerative diseases

(2009) Bess Frost et al.   NATURE REVIEWS NEUROSCIENCE

The effect of mutant SOD1 dismutase activity on non-cell autonomous degeneration in familial amyotrophic lateral sclerosis

(2009) Lijun Wang et al.   NEUROBIOLOGY OF DISEASE

Multicenter Analysis of Glucocerebrosidase Mutations in Parkinson's Disease

(2009) E. Sidransky et al.   NEW ENGLAND JOURNAL OF MEDICINE

Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

(2008) Masato Hasegawa et al.   ANNALS OF NEUROLOGY

Skeletal Muscle Is a Primary Target of SOD1G93A-Mediated Toxicity

(2008) Gabriella Dobrowolny et al.   Cell Metabolism

Abnormal phosphorylation of Ser409/410 of TDP-43 in FTLD-U and ALS

(2008) Yuki Inukai et al.   FEBS LETTERS

ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1

(2008) H. Nishitoh et al.   GENES & DEVELOPMENT

The AβCs of Aβ-cleaving Proteases

(2008) Malcolm A. Leissring   JOURNAL OF BIOLOGICAL CHEMISTRY

Polo-like Kinase 2 (PLK2) Phosphorylates α-Synuclein at Serine 129 in Central Nervous System

(2008) Kelly J. Inglis et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Hsp104 Targets Multiple Intermediates on the Amyloid Pathway and Suppresses the Seeding Capacity of Aβ Fibrils and Protofibrils

(2008) Muriel Arimon et al.   JOURNAL OF MOLECULAR BIOLOGY

α-Synuclein–induced Aggregation of Cytoplasmic Vesicles in Saccharomyces cerevisiae

(2008) James H. Soper et al.   MOLECULAR BIOLOGY OF THE CELL

Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis

(2008) Koji Yamanaka et al.   NATURE NEUROSCIENCE

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

(2008) Evangelia Emmanouilidou et al.   NEUROBIOLOGY OF AGING

The Ubiquitin-Proteasome Pathway in Huntington's Disease

(2008) Siddhartha Mitra et al.   TheScientificWorldJOURNAL