The role of amyloid oligomers in neurodegenerative pathologies

Targeting Small Molecule Delivery to the Brain and Spinal Cord via Intranasal Administration of Rabies Virus Glycoprotein (RVG29)-Modified PLGA Nanoparticles

(2020) Eugene P. Chung et al.   Pharmaceutics

Dynamics of oligomer and amyloid fibril formation by yeast prion Sup35 observed by high-speed atomic force microscopy

(2020) Hiroki Konno et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

In vitro modeling of the neurovascular unit: advances in the field

(2020) Aditya Bhalerao et al.   Fluids and Barriers of the CNS

Modelling neurodegenerative disease using brain organoids

(2020) Selina Wray   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Neurotransmitter-derived lipidoids (NT-lipidoids) for enhanced brain delivery through intravenous injection

(2020) Feihe Ma et al.   Science Advances

Aβ, tau, α-synuclein… are they prions, prion-like proteins, or what?

(2019) Hasier Eraña   Prion

Microtubules Deform the Nuclear Membrane and Disrupt Nucleocytoplasmic Transport in Tau-Mediated Frontotemporal Dementia

(2019) Francesco Paonessa et al.   Cell Reports

Detection of TAR DNA-binding protein 43 (TDP-43) oligomers as initial intermediate species during aggregate formation

(2019) Rachel L. French et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Increased BBB permeability contributes to EGCG-caused cognitive function improvement in natural aging rats: pharmacokinetic and distribution analyses

(2019) Bin-bin Wei et al.   ACTA PHARMACOLOGICA SINICA

The debated toxic role of aggregated TDP-43 in amyotrophic lateral sclerosis: a resolution in sight?

(2019) Rudolf C Hergesheimer et al.   BRAIN

Secondary nucleation and elongation occur at different sites on Alzheimer’s amyloid-β aggregates

(2019) Tom Scheidt et al.   Science Advances

The metastability of the proteome of spinal motor neurons underlies their selective vulnerability in ALS

(2019) Justin J. Yerbury et al.   NEUROSCIENCE LETTERS

E46K mutant α-synuclein is more degradation resistant and exhibits greater toxic effects than wild-type α-synuclein in Drosophila models of Parkinson's disease

(2019) Ryusuke Sakai et al.   PLoS One

Ageing as a risk factor for neurodegenerative disease

(2019) Yujun Hou et al.   Nature Reviews Neurology

The growth of amyloid fibrils: rates and mechanisms

(2019) Alexander K. Buell   BIOCHEMICAL JOURNAL

Mitochondrial dysfunction and neurodegenerative proteinopathies: mechanisms and prospects for therapeutic intervention

(2018) Thomas Briston et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Secondary nucleation in amyloid formation

(2018) Mattias Törnquist et al.   CHEMICAL COMMUNICATIONS

Integration of multi-omics datasets enables molecular classification of COPD

(2018) Chuan-Xing Li et al.   EUROPEAN RESPIRATORY JOURNAL

Angubindin-1 opens the blood–brain barrier in vivo for delivery of antisense oligonucleotide to the central nervous system

(2018) Satoshi Zeniya et al.   JOURNAL OF CONTROLLED RELEASE

Novel genes associated with amyotrophic lateral sclerosis: diagnostic and clinical implications

(2018) Ruth Chia et al.   LANCET NEUROLOGY

Cholesterol catalyses Aβ42 aggregation through a heterogeneous nucleation pathway in the presence of lipid membranes

(2018) Johnny Habchi et al.   Nature Chemistry

Autophagy as a common pathway in amyotrophic lateral sclerosis

(2018) Dao K.H. Nguyen et al.   NEUROSCIENCE LETTERS

Large SOD1 aggregates, unlike trimeric SOD1, do not impact cell viability in a model of amyotrophic lateral sclerosis

(2018) Cheng Zhu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Origin of metastable oligomers and their effects on amyloid fibril self-assembly

(2018) Filip Hasecke et al.   Chemical Science

Current Strategies for Brain Drug Delivery

(2018) Xiaowei Dong   Theranostics

The LepR-mediated leptin transport across brain barriers controls food reward

(2018) Alessandro Di Spiezio et al.   Molecular Metabolism

C9orf72-mediated ALS and FTD: multiple pathways to disease

(2018) Rubika Balendra et al.   Nature Reviews Neurology

Novel antibodies reveal presynaptic localization of C9orf72 protein and reduced protein levels in C9orf72 mutation carriers

(2018) Petra Frick et al.   Acta Neuropathologica Communications

Congo Red and amyloids: history and relationship

(2018) Elmira I. Yakupova et al.   BIOSCIENCE REPORTS

The Implications of Autophagy in Alzheimer's Disease

(2018) Tadanori Hamano et al.   Current Alzheimer Research

An oligomer-specific antibody improved motor function and attenuated neuropathology in the SOD1-G93A transgenic mouse model of ALS

(2018) Quan-xiu Dong et al.   INTERNATIONAL IMMUNOPHARMACOLOGY

Global, regional, and national burden of Alzheimer's disease and other dementias, 1990–2016: a systematic analysis for the Global Burden of Disease Study 2016

(2018) Emma Nichols et al.   LANCET NEUROLOGY

Blood–brain-barrier organoids for investigating the permeability of CNS therapeutics

(2018) Sonja Bergmann et al.   Nature Protocols

Quantitative assessment of the degradation of aggregated TDP-43 mediated by the ubiquitin proteasome system and macroautophagy

(2017) Roberta Cascella et al.   FASEB JOURNAL

Synergistic effects of negatively charged hydrophobic nanoparticles and (−)-epigallocatechin-3-gallate on inhibiting amyloid β-protein aggregation

(2017) Hongchen Liu et al.   JOURNAL OF COLLOID AND INTERFACE SCIENCE

Angubindin-1, a novel paracellular absorption enhancer acting at the tricellular tight junction

(2017) Susanne M. Krug et al.   JOURNAL OF CONTROLLED RELEASE

From Aβ Filament to Fibril: Molecular Mechanism of Surface-Activated Secondary Nucleation from All-Atom MD Simulations

(2017) Nadine Schwierz et al.   JOURNAL OF PHYSICAL CHEMISTRY B

Targeting the Central Nervous System (CNS): A Review of Rabies Virus-Targeting Strategies

(2017) Mira Oswald et al.   MOLECULAR PHARMACEUTICS

A Basic ApoE-Based Peptide Mediator to Deliver Proteins across the Blood-Brain Barrier: Long-Term Efficacy, Toxicity, and Mechanism

(2017) Yu Meng et al.   MOLECULAR THERAPY

Atomic structure of a toxic, oligomeric segment of SOD1 linked to amyotrophic lateral sclerosis (ALS)

(2017) Smriti Sangwan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Role of Lipids Interacting with α-Synuclein in the Pathogenesis of Parkinson’s Disease

(2017) Céline Galvagnion   Journal of Parkinsons Disease

Reversible unfolding of infectious prion assemblies reveals the existence of an oligomeric elementary brick

(2017) Angélique Igel-Egalon et al.   PLoS Pathogens

Chaperones as Suppressors of Protein Misfolded Oligomer Toxicity

(2017) Benedetta Mannini et al.   Frontiers in Molecular Neuroscience

Neuro-degeneration profile of Alzheimer's patients: A brain morphometry study

(2017) Silvio Ramos Bernardes da Silva Filho et al.   NeuroImage-Clinical

Generation of a Motor Nerve Organoid with Human Stem Cell-Derived Neurons

(2017) Jiro Kawada et al.   Stem Cell Reports

Suppression of Map Kinases Inhibits Microglial Activation and Attenuates Neuronal Cell Death Induced by α-Synuclein Protofibrils

(2017) H. Wilms et al.   INTERNATIONAL JOURNAL OF IMMUNOPATHOLOGY AND PHARMACOLOGY

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

The C9orf72 protein interacts with Rab1a and the ULK1 complex to regulate initiation of autophagy

(2016) Christopher P Webster et al.   EMBO JOURNAL

Clusterin Binds to Aβ1–42Oligomers with High Affinity and Interferes with Peptide Aggregation by Inhibiting Primary and Secondary Nucleation

(2016) Marten Beeg et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Membrane-bound tetramer and trimer Aβ oligomeric species correlate with toxicity towards cultured neurons

(2016) Metta K. Jana et al.   JOURNAL OF NEUROCHEMISTRY

Tau Oligomers Derived from Traumatic Brain Injury Cause Cognitive Impairment and Accelerate Onset of Pathology in Htau Mice

(2016) Julia Gerson et al.   JOURNAL OF NEUROTRAUMA

C9orf72 binds SMCR8, localizes to lysosomes, and regulates mTORC1 signaling

(2016) Joseph Amick et al.   MOLECULAR BIOLOGY OF THE CELL

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

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

Structural insights into the multi-determinant aggregation of TDP-43 in motor neuron-like cells

(2016) F. Bozzo et al.   NEUROBIOLOGY OF DISEASE

Phosphorylated TDP-43 becomes resistant to cleavage by calpain: A regulatory role for phosphorylation in TDP-43 pathology of ALS/FTLD

(2016) Takenari Yamashita et al.   NEUROSCIENCE RESEARCH

Holdase activity of secreted Hsp70 masks amyloid-β42 neurotoxicity inDrosophila

(2016) Pedro Fernandez-Funez et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Systematic development of small molecules to inhibit specific microscopic steps of Aβ42 aggregation in Alzheimer’s disease

(2016) Johnny Habchi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TDP-43/FUS in motor neuron disease: Complexity and challenges

(2016) Erika N. Guerrero et al.   PROGRESS IN NEUROBIOLOGY

C9orf72 is required for proper macrophage and microglial function in mice

(2016) J. G. ORourke et al.   SCIENCE

C9orf72 Hexanucleotide Expansions Are Associated with Altered Endoplasmic Reticulum Calcium Homeostasis and Stress Granule Formation in Induced Pluripotent Stem Cell-Derived Neurons from Patients with Amyotrophic Lateral Sclerosis and Frontotemporal Demen

(2016) Ruxandra Dafinca et al.   STEM CELLS

FUS Mislocalization and Vulnerability to DNA Damage in ALS Patients Derived hiPSCs and Aging Motoneurons

(2016) Julia Higelin et al.   Frontiers in Cellular Neuroscience

Tau Structures

(2016) Jesus Avila et al.   Frontiers in Aging Neuroscience

Electrostatic Repulsion Governs TDP-43 C-terminal Domain Aggregation

(2016) Miguel Mompeán et al.   PLOS BIOLOGY

ALS-Causing Mutations Significantly Perturb the Self-Assembly and Interaction with Nucleic Acid of the Intrinsically Disordered Prion-Like Domain of TDP-43

(2016) Liangzhong Lim et al.   PLOS BIOLOGY

Copper-induced structural conversion templates prion protein oligomerization and neurotoxicity

(2016) C.-F. Yen et al.   Science Advances

Pathogenesis of FUS-associated ALS and FTD: insights from rodent models

(2016) Matthew Nolan et al.   Acta Neuropathologica Communications

Specific Targeting of Tau Oligomers in Htau Mice Prevents Cognitive Impairment and Tau Toxicity Following Injection with Brain-Derived Tau Oligomeric Seeds

(2016) Diana L. Castillo-Carranza et al.   JOURNAL OF ALZHEIMERS DISEASE

Alzheimer’s Protective A2T Mutation Changes the Conformational Landscape of the Aβ1–42 Monomer Differently Than Does the A2V Mutation

(2015) Payel Das et al.   BIOPHYSICAL JOURNAL

Exogenous α-synuclein induces toll-like receptor 4 dependent inflammatory responses in astrocytes

(2015) Emmy H. Rannikko et al.   BMC NEUROSCIENCE

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

(2015) Avinash Patel et al.   CELL

Structural, morphological, and functional diversity of amyloid oligomers

(2015) Leonid Breydo et al.   FEBS LETTERS

Subcellular localization and RNAs determine FUS architecture in different cellular compartments

(2015) Liuqing Yang et al.   HUMAN MOLECULAR GENETICS

Brain region-dependent differential expression of alpha-synuclein

(2015) Katsutoshi Taguchi et al.   JOURNAL OF COMPARATIVE NEUROLOGY

Oligomer Formation of Toxic and Functional Amyloid Peptides Studied with Atomistic Simulations

(2015) Martín Carballo-Pacheco et al.   JOURNAL OF PHYSICAL CHEMISTRY B

Amyloid β-Protein Assembly: The Effect of Molecular Tweezers CLR01 and CLR03

(2015) Xueyun Zheng et al.   JOURNAL OF PHYSICAL CHEMISTRY B

Parkin Protects Against Misfolded SOD1 Toxicity by Promoting Its Aggresome Formation and Autophagic Clearance

(2015) Cheryl Yung et al.   MOLECULAR NEUROBIOLOGY

Active immunization against alpha-synuclein ameliorates the degenerative pathology and prevents demyelination in a model of multiple system atrophy

(2015) Markus Mandler et al.   Molecular Neurodegeneration

A molecular chaperone breaks the catalytic cycle that generates toxic Aβ oligomers

(2015) Samuel I A Cohen et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Isolation and characterization of antibody fragments selective for toxic oligomeric tau

(2015) Huilai Tian et al.   NEUROBIOLOGY OF AGING

Nonnative SOD1 trimer is toxic to motor neurons in a model of amyotrophic lateral sclerosis

(2015) Elizabeth A. Proctor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA

(2015) A. C. Woerner et al.   SCIENCE

Supersaturation is a major driving force for protein aggregation in neurodegenerative diseases

(2015) Prajwal Ciryam et al.   TRENDS IN PHARMACOLOGICAL SCIENCES

An acetylation switch controls TDP-43 function and aggregation propensity

(2015) Todd J. Cohen et al.   Nature Communications

A molecular chaperone breaks the catalytic cycle that generates toxic Aβ oligomers

(2015) Samuel I A Cohen et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Structure based aggregation studies reveal the presence of helix-rich intermediate during α-Synuclein aggregation

(2015) Dhiman Ghosh et al.   Scientific Reports

Modulation of SOD1 Subcellular Localization by Transfection with Wild- or Mutant-type SOD1 in Primary Neuron and Astrocyte Cultures from ALS Mice

(2015) Do-Yeon Lee et al.   Experimental Neurobiology

Post-Translational Modifications Promote Formation of SOD1 Oligomers with Potential Toxicity in ALS

(2015) Nikolay V. Dokholyan et al.   BIOPHYSICAL JOURNAL

Toxicity of Protein Oligomers Is Rationalized by a Function Combining Size and Surface Hydrophobicity

(2014) Benedetta Mannini et al.   ACS Chemical Biology

Co-existence of Two Different α-Synuclein Oligomers with Different Core Structures Determined by Hydrogen/Deuterium Exchange Mass Spectrometry

(2014) Wojciech Paslawski et al.   ANGEWANDTE CHEMIE-INTERNATIONAL EDITION

Mechanism of Amyloid-β Fibril Elongation

(2014) Thomas Gurry et al.   BIOCHEMISTRY

Multistep process of FUS aggregation in the cell cytoplasm involves RNA-dependent and RNA-independent mechanisms

(2014) Tatyana A. Shelkovnikova et al.   HUMAN MOLECULAR GENETICS

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

Differential Effects of Synaptic and Extrasynaptic NMDA Receptors on A -Induced Nitric Oxide Production in Cerebrocortical Neurons

(2014) E. Molokanova et al.   JOURNAL OF NEUROSCIENCE

Surface Effects on Aggregation Kinetics of Amyloidogenic Peptides

(2014) Robert Vácha et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Amyloid Oligomers and Protofibrils, but Not Filaments, Self-Replicate from Native Lysozyme

(2014) Mentor Mulaj et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Single-molecule tracking of tau reveals fast kiss-and-hop interaction with microtubules in living neurons

(2014) Dennis Janning et al.   MOLECULAR BIOLOGY OF THE CELL

C9orf72 nucleotide repeat structures initiate molecular cascades of disease

(2014) Aaron R. Haeusler et al.   NATURE

The role of FUS gene variants in neurodegenerative diseases

(2014) Hao Deng et al.   Nature Reviews Neurology

Immunotherapy targeting α-synuclein protofibrils reduced pathology in (Thy-1)-h[A30P] α-synuclein mice

(2014) Veronica Lindström et al.   NEUROBIOLOGY OF DISEASE

Discovery of a Biomarker and Lead Small Molecules to Target r(GGGGCC)-Associated Defects in c9FTD/ALS

(2014) Zhaoming Su et al.   NEURON

­Classifying prion and prion-like phenomena

(2014) Djamel Harbi et al.   Prion

Self-assembled FUS binds active chromatin and regulates gene transcription

(2014) Liuqing Yang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A plural role for lipids in motor neuron diseases: energy, signaling and structure

(2014) Florent Schmitt et al.   Frontiers in Cellular Neuroscience

Full-length TDP-43 forms toxic amyloid oligomers that are present in frontotemporal lobar dementia-TDP patients

(2014) Yu-Sheng Fang et al.   Nature Communications

Safety and pharmacological characterization of the molecular tweezer CLR01 – a broad-spectrum inhibitor of amyloid proteins’ toxicity

(2014) Aida Attar et al.   BMC Pharmacology & Toxicology

Prion protein oligomer and its neurotoxicity

(2013) P. Huang et al.   ACTA BIOCHIMICA ET BIOPHYSICA SINICA

Bidirectional transcripts of the expanded C9orf72 hexanucleotide repeat are translated into aggregating dipeptide repeat proteins

(2013) Kohji Mori et al.   ACTA NEUROPATHOLOGICA

Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons

(2013) Sandra Almeida et al.   ACTA NEUROPATHOLOGICA

Inclusions in frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP) and amyotrophic lateral sclerosis (ALS), but not FTLD with FUS proteinopathy (FTLD-FUS), have properties of amyloid

(2013) Eileen H. Bigio et al.   ACTA NEUROPATHOLOGICA

An N-terminal Nuclear Export Signal Regulates Trafficking and Aggregation of Huntingtin (Htt) Protein Exon 1

(2013) Zhiqiang Zheng et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Monoclonal antibodies selective for α-synuclein oligomers/protofibrils recognize brain pathology in Lewy body disorders and α-synuclein transgenic mice with the disease-causing A30P mutation

(2013) Therese Fagerqvist et al.   JOURNAL OF NEUROCHEMISTRY

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

(2013) Shuo-Chien Ling et al.   NEURON

The Omega-3 Fatty Acid Eicosapentaenoic Acid Accelerates Disease Progression in a Model of Amyotrophic Lateral Sclerosis

(2013) Ping K. Yip et al.   PLoS One

Conformational Stability of Fibrillar Amyloid-Beta Oligomers via Protofilament Pair Formation – A Systematic Computational Study

(2013) Anna Kahler et al.   PLoS One

A  induces astrocytic glutamate release, extrasynaptic NMDA receptor activation, and synaptic loss

(2013) M. Talantova 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

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

(2013) Julia Leitman et al.   Nature Communications

Widespread Aggregation and Neurodegenerative Diseases Are Associated with Supersaturated Proteins

(2013) Prajwal Ciryam et al.   Cell Reports

Deciphering amyotrophic lateral sclerosis: What phenotype, neuropathology and genetics are telling us about pathogenesis

(2013) John Ravits et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Synapse-Binding Subpopulations of Aβ Oligomers Sensitive to Peptide Assembly Blockers and scFv Antibodies

(2012) Pauline T. Velasco et al.   ACS Chemical Neuroscience

Amyloid fibril protein nomenclature: 2012 recommendations from the Nomenclature Committee of the International Society of Amyloidosis

(2012) Jean D. Sipe et al.   AMYLOID-JOURNAL OF PROTEIN FOLDING DISORDERS

Protein Misfolded Oligomers: Experimental Approaches, Mechanism of Formation, and Structure-Toxicity Relationships

(2012) Francesco Bemporad et al.   CHEMISTRY & BIOLOGY

Identification of oligomers at early stages of tau aggregation in Alzheimer's disease

(2012) Cristian A. Lasagna-Reeves et al.   FASEB JOURNAL

Toll-like receptor 4 is required for α-synuclein dependent activation of microglia and astroglia

(2012) Lisa Fellner et al.   GLIA

BRICHOS Domains Efficiently Delay Fibrillation of Amyloid β-Peptide

(2012) Hanna Willander et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Swedish Alzheimer Mutation Induces Mitochondrial Dysfunction Mediated by HSP60 Mislocalization of Amyloid Precursor Protein (APP) and Beta-Amyloid

(2012) Ken Carlson Walls et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Correlation of Alzheimer Disease Neuropathologic Changes With Cognitive Status: A Review of the Literature

(2012) Peter T. Nelson et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Apolipoprotein E, Especially Apolipoprotein E4, Increases the Oligomerization of Amyloid   Peptide

(2012) T. Hashimoto et al.   JOURNAL OF NEUROSCIENCE

Mitochondria, oxidative stress and neurodegeneration

(2012) Antonio Federico et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Heat shock protein 90 in neurodegenerative diseases

(2012) Wenjie Luo et al.   Molecular Neurodegeneration

Molecular mechanisms used by chaperones to reduce the toxicity of aberrant protein oligomers

(2012) B. Mannini et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Out-of-register  -sheets suggest a pathway to toxic amyloid aggregates

(2012) C. Liu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The self-interaction of native TDP-43 C terminus inhibits its degradation and contributes to early proteinopathies

(2012) I.-Fan Wang et al.   Nature Communications

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

(2012) Pietro Fratta et al.   Scientific Reports

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

(2011) Heidi Olzscha et al.   CELL

Amyloid deposits: Protection against toxic protein species?

(2011) Sebastian Treusch et al.   CELL CYCLE

Redox signalling directly regulates TDP-43 via cysteine oxidation and disulphide cross-linking

(2011) Todd J Cohen et al.   EMBO JOURNAL

Regulation of Autophagy by Neuropathological Protein TDP-43

(2011) Jayarama Krishnan Bose et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Characterization of Prefibrillar Tau Oligomersin Vitroand in Alzheimer Disease

(2011) Kristina R. Patterson et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Lysine-Specific Molecular Tweezers Are Broad-Spectrum Inhibitors of Assembly and Toxicity of Amyloid Proteins

(2011) Sharmistha Sinha et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Sequestration of Toxic Oligomers by HspB1 as a Cytoprotective Mechanism

(2011) J. Ojha et al.   MOLECULAR AND CELLULAR BIOLOGY

Amyloid beta protein-induced zinc sequestration leads to synaptic loss via dysregulation of the ProSAP2/Shank3 scaffold

(2011) Andreas M Grabrucker et al.   Molecular Neurodegeneration

Tau oligomers impair memory and induce synaptic and mitochondrial dysfunction in wild-type mice

(2011) Cristian A Lasagna-Reeves et al.   Molecular Neurodegeneration

Clinical genetics of amyotrophic lateral sclerosis: what do we really know?

(2011) Peter M. Andersen et al.   Nature Reviews Neurology

An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity

(2011) Weirui Guo et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

The influence and the mechanism of docosahexaenoic acid on a mouse model of Parkinson’s disease

(2011) Ozlem Ozsoy et al.   NEUROCHEMISTRY INTERNATIONAL

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

(2011) Mariely DeJesus-Hernandez et al.   NEURON

DHA Improves Cognition and Prevents Dysfunction of Entorhinal Cortex Neurons in 3xTg-AD Mice

(2011) Dany Arsenault et al.   PLoS One

Human apoE Isoforms Differentially Regulate Brain Amyloid-  Peptide Clearance

(2011) J. M. Castellano et al.   Science Translational Medicine

Molecular Determinants and Genetic Modifiers of Aggregation and Toxicity for the ALS Disease Protein FUS/TLS

(2011) Zhihui Sun et al.   PLOS BIOLOGY

ANS Binding Reveals Common Features of Cytotoxic Amyloid Species

(2010) Benedetta Bolognesi et al.   ACS Chemical Biology

FUS Mutations in Familial Amyotrophic Lateral Sclerosis in the Netherlands

(2010) Ewout J. N. Groen et al.   ARCHIVES OF NEUROLOGY

Preparation and Characterization of Neurotoxic Tau Oligomers

(2010) Cristian A. Lasagna-Reeves et al.   BIOCHEMISTRY

Retardation of Aβ Fibril Formation by Phospholipid Vesicles Depends on Membrane Phase Behavior

(2010) Erik Hellstrand et al.   BIOPHYSICAL JOURNAL

Striatal and white matter predictors of estimated diagnosis for Huntington disease

(2010) Jane S. Paulsen et al.   BRAIN RESEARCH BULLETIN

Neurotoxicity of Alzheimer's disease Aβ peptides is induced by small changes in the Aβ42 to Aβ40 ratio

(2010) Inna Kuperstein et al.   EMBO JOURNAL

ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import

(2010) Dorothee Dormann et al.   EMBO JOURNAL

Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules

(2010) Daryl A. Bosco et al.   HUMAN MOLECULAR GENETICS

Aβ42 Neurotoxicity Is Mediated by Ongoing Nucleated Polymerization Process Rather than by Discrete Aβ42 Species

(2010) Asad Jan et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Prion-like disorders: blurring the divide between transmissibility and infectivity

(2010) M. Cushman et al.   JOURNAL OF CELL SCIENCE

Expression of  -Amyloid Induced Age-Dependent Presynaptic and Axonal Changes in Drosophila

(2010) X.-L. Zhao et al.   JOURNAL OF NEUROSCIENCE

Cytoplasmic Mislocalization of TDP-43 Is Toxic to Neurons and Enhanced by a Mutation Associated with Familial Amyotrophic Lateral Sclerosis

(2010) S. J. Barmada et al.   JOURNAL OF NEUROSCIENCE

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

(2010) Ian RA Mackenzie et al.   LANCET NEUROLOGY

Preparation and characterization of toxic Aβ aggregates for structural and functional studies in Alzheimer's disease research

(2010) Asad Jan et al.   Nature Protocols

Tau Mislocalization to Dendritic Spines Mediates Synaptic Dysfunction Independently of Neurodegeneration

(2010) Brian R. Hoover et al.   NEURON

Decreased Clearance of CNS  -Amyloid in Alzheimer's Disease

(2010) K. G. Mawuenyega et al.   SCIENCE

Biology of Amyloid: Structure, Function, and Regulation

(2010) Jason Greenwald et al.   STRUCTURE

Distinct Brain Volume Changes Correlating with Clinical Stage, Disease Progression Rate, Mutation Size, and Age at Onset Prediction as Early Biomarkers of Brain Atrophy in Huntington's Disease

(2009) Ferdinando Squitieri et al.   CNS Neuroscience & Therapeutics

Proteomic characterization of lipid raft proteins in amyotrophic lateral sclerosis mouse spinal cord

(2009) Jianjun Zhai et al.   FEBS Journal

Fibrillar Oligomers Nucleate the Oligomerization of Monomeric Amyloid β but Do Not Seed Fibril Formation

(2009) Jessica W. Wu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Genome-wide association study identifies variants at CLU and CR1 associated with Alzheimer's disease

(2009) Jean-Charles Lambert et al.   NATURE GENETICS

Genome-wide association study identifies variants at CLU and PICALM associated with Alzheimer's disease

(2009) Denise Harold et al.   NATURE GENETICS

Analysis of FUS gene mutation in familial amyotrophic lateral sclerosis within an Italian cohort

(2009) N. Ticozzi et al.   NEUROLOGY

Mitochondrial Mislocalization Underlies Aβ42-Induced Neuronal Dysfunction in a Drosophila Model of Alzheimer's Disease

(2009) Kanae Iijima-Ando et al.   PLoS One

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

Structure-neurotoxicity relationships of amyloid  -protein oligomers

(2009) K. Ono et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Oligomeric amyloid   associates with postsynaptic densities and correlates with excitatory synapse loss near senile plaques

(2009) R. M. Koffie et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

(2009) T. J. Kwiatkowski et al.   SCIENCE

Functional Amyloids As Natural Storage of Peptide Hormones in Pituitary Secretory Granules

(2009) S. K. Maji et al.   SCIENCE

Evidence of Multisystem Disorder in Whole-Brain Map of Pathological TDP-43 in Amyotrophic Lateral Sclerosis

(2008) Felix Geser et al.   ARCHIVES OF NEUROLOGY

Hsp104 antagonizes α-synuclein aggregation and reduces dopaminergic degeneration in a rat model of Parkinson disease

(2008) Christophe Lo Bianco et al.   JOURNAL OF CLINICAL INVESTIGATION

apoE isoform–specific disruption of amyloid β peptide clearance from mouse brain

(2008) Rashid Deane et al.   JOURNAL OF CLINICAL INVESTIGATION

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

Thirty years of Alzheimer's disease genetics: the implications of systematic meta-analyses

(2008) Lars Bertram et al.   NATURE REVIEWS NEUROSCIENCE

EGCG redirects amyloidogenic polypeptides into unstructured, off-pathway oligomers

(2008) Dagmar E Ehrnhoefer et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Dyslipidemia is a protective factor in amyotrophic lateral sclerosis

(2008) L. Dupuis et al.   NEUROLOGY

Alzheimer's disease-type neuronal tau hyperphosphorylation induced by Aβ oligomers

(2007) Fernanda G. De Felice et al.   NEUROBIOLOGY OF AGING