Animal models of neurodegenerative diseases

Mouse models of ALS: Past, present and future

(2018) Cathleen Lutz   BRAIN RESEARCH

Modeling human diseases with induced pluripotent stem cells: from 2D to 3D and beyond

(2018) Chun Liu et al.   DEVELOPMENT

Poly(GR) impairs protein translation and stress granule dynamics in C9orf72-associated frontotemporal dementia and amyotrophic lateral sclerosis

(2018) Yong-Jie Zhang et al.   NATURE MEDICINE

Block of A1 astrocyte conversion by microglia is neuroprotective in models of Parkinson’s disease

(2018) Seung Pil Yun et al.   NATURE MEDICINE

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

(2018) Yingxiao Shi et al.   NATURE MEDICINE

TDP-43 gains function due to perturbed autoregulation in a Tardbp knock-in mouse model of ALS-FTD

(2018) Matthew A. White et al.   NATURE NEUROSCIENCE

Microglia-mediated recovery from ALS-relevant motor neuron degeneration in a mouse model of TDP-43 proteinopathy

(2018) Krista J. Spiller et al.   NATURE NEUROSCIENCE

Robust kinase- and age-dependent dopaminergic and norepinephrine neurodegeneration in LRRK2 G2019S transgenic mice

(2018) Yulan Xiong et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Genetic risk for Alzheimer’s disease is concentrated in specific macrophage and microglial transcriptional networks

(2018) Katherine E. Tansey et al.   Genome Medicine

Clinical approaches to the development of a neuroprotective therapy for PD

(2017) C.W. Olanow et al.   EXPERIMENTAL NEUROLOGY

Trumping neurodegeneration: Targeting common pathways regulated by autosomal recessive Parkinson's disease genes

(2017) Laura Scott et al.   EXPERIMENTAL NEUROLOGY

Distinct differences in prion-like seeding and aggregation between Tau protein variants provide mechanistic insights into tauopathies

(2017) Kevin H. Strang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The prion model for progression and diversity of neurodegenerative diseases

(2017) Barbara E Stopschinski et al.   LANCET NEUROLOGY

A meta-analysis of genome-wide association studies identifies 17 new Parkinson's disease risk loci

(2017) Diana Chang et al.   NATURE GENETICS

Amyloid-β plaques enhance Alzheimer's brain tau-seeded pathologies by facilitating neuritic plaque tau aggregation

(2017) Zhuohao He et al.   NATURE MEDICINE

Comparative transcriptomics in human and mouse

(2017) Alessandra Breschi et al.   NATURE REVIEWS GENETICS

Animal models of α-synucleinopathy for Parkinson disease drug development

(2017) James B. Koprich et al.   NATURE REVIEWS NEUROSCIENCE

Age-Dependent Dopaminergic Neurodegeneration and Impairment of the Autophagy-Lysosomal Pathway in LRRK -Deficient Mice

(2017) Emilie Giaime et al.   NEURON

Tau Pathology Induces Excitatory Neuron Loss, Grid Cell Dysfunction, and Spatial Memory Deficits Reminiscent of Early Alzheimer’s Disease

(2017) Hongjun Fu et al.   NEURON

Mouse models of frontotemporal dementia: A comparison of phenotypes with clinical symptomatology

(2017) Rebekah M. Ahmed et al.   NEUROSCIENCE AND BIOBEHAVIORAL REVIEWS

Pathogenic determinants and mechanisms of ALS/FTD linked to hexanucleotide repeat expansions in the C9orf72 gene

(2017) Xinmei Wen et al.   NEUROSCIENCE LETTERS

PINK1 Primes Parkin-Mediated Ubiquitination of PARIS in Dopaminergic Neuronal Survival

(2017) Yunjong Lee 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

Are rodent models of Parkinson’s disease behaving as they should?

(2017) Siv Vingill et al.   BEHAVIOURAL BRAIN RESEARCH

Toward more predictive genetic mouse models of Alzheimer's disease

(2016) Kristen D. Onos et al.   BRAIN RESEARCH BULLETIN

Statistical tests, P values, confidence intervals, and power: a guide to misinterpretations

(2016) Sander Greenland et al.   EUROPEAN JOURNAL OF EPIDEMIOLOGY

Mammalian prions and their wider relevance in neurodegenerative diseases

(2016) John Collinge   NATURE

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins

(2016) Yong-Jie Zhang et al.   NATURE NEUROSCIENCE

A/T/N: An unbiased descriptive classification scheme for Alzheimer disease biomarkers

(2016) Clifford R. Jack et al.   NEUROLOGY

C9orf72 BAC Mouse Model with Motor Deficits and Neurodegenerative Features of ALS/FTD

(2016) Yuanjing Liu et al.   NEURON

Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs

(2016) Jie Jiang et al.   NEURON

Tau Prion Strains Dictate Patterns of Cell Pathology, Progression Rate, and Regional Vulnerability In Vivo

(2016) Sarah K. Kaufman et al.   NEURON

Challenges in detecting disease modification in Parkinson's disease clinical trials

(2016) Dilan Athauda et al.   PARKINSONISM & RELATED DISORDERS

Motor neuron disease, TDP-43 pathology, and memory deficits in mice expressing ALS–FTD-linkedUBQLN2mutations

(2016) Nhat T. T. Le 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

C9orf72 is required for proper macrophage and microglial function in mice

(2016) J. G. ORourke et al.   SCIENCE

Loss-of-function mutations in the C9ORF72 mouse ortholog cause fatal autoimmune disease

(2016) Aaron Burberry et al.   Science Translational Medicine

α-Synuclein-Based Animal Models of Parkinson's Disease: Challenges and Opportunities in a New Era

(2016) Naomi P. Visanji et al.   TRENDS IN NEUROSCIENCES

From animal models to human disease: a genetic approach for personalized medicine in ALS

(2016) Vincent Picher-Martel et al.   Acta Neuropathologica Communications

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

(2016) Matthew Nolan et al.   Acta Neuropathologica Communications

C9orf72 ablation causes immune dysregulation characterized by leukocyte expansion, autoantibody production and glomerulonephropathy in mice

(2016) Amanda Atanasio et al.   Scientific Reports

Non-prion-type transmission in A53T α-synuclein transgenic mice: a normal component of spinal homogenates from naïve non-transgenic mice induces robust α-synuclein pathology

(2015) Amanda N. Sacino et al.   ACTA NEUROPATHOLOGICA

Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43

(2015) Adam K. Walker et al.   ACTA NEUROPATHOLOGICA

Neurodegenerative Diseases: Expanding the Prion Concept

(2015) Lary C. Walker et al.   Annual Review of Neuroscience

FTD and ALS—translating mouse studies into clinical trials

(2015) Lars M. Ittner et al.   Nature Reviews Neurology

Spreading of pathology in neurodegenerative diseases: a focus on human studies

(2015) Johannes Brettschneider et al.   NATURE REVIEWS NEUROSCIENCE

Adenoviral-mediated expression of G2019S LRRK2 induces striatal pathology in a kinase-dependent manner in a rat model of Parkinson's disease

(2015) Elpida Tsika et al.   NEUROBIOLOGY OF DISEASE

Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice

(2015) Owen M. Peters et al.   NEURON

C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD

(2015) Jacqueline G. O’Rourke et al.   NEURON

Clinical Trial Designs in Amyotrophic Lateral Sclerosis: Does One Design Fit All?

(2015) Katharine A. Nicholson et al.   Neurotherapeutics

C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits

(2015) J. Chew et al.   SCIENCE

Four pioneers of L-dopa treatment: Arvid Carlsson, Oleh Hornykiewicz, George Cotzias, and Melvin Yahr

(2014) Andrew J. Lees et al.   MOVEMENT DISORDERS

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

(2014) Jing L Guo et al.   NATURE MEDICINE

Single App knock-in mouse models of Alzheimer's disease

(2014) Takashi Saito et al.   NATURE NEUROSCIENCE

Serial propagation of distinct strains of A  prions from Alzheimer's disease patients

(2014) J. C. Watts et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The A4 Study: Stopping AD Before Symptoms Begin?

(2014) R. A. Sperling et al.   Science Translational Medicine

Parkinson’s disease: animal models and dopaminergic cell vulnerability

(2014) Javier Blesa et al.   Frontiers in Neuroanatomy

Predicting success: Optimizing phase II ALS trials for the transition to phase III

(2014) James D. Berry et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Modeling neurodegenerative diseases in Caenorhabditis elegans

(2013) Jia Li et al.   EXPERIMENTAL NEUROLOGY

Thinking laterally about neurodegenerative proteinopathies

(2013) Todd E. Golde et al.   JOURNAL OF CLINICAL INVESTIGATION

An antisense oligonucleotide against SOD1 delivered intrathecally for patients with SOD1 familial amyotrophic lateral sclerosis: a phase 1, randomised, first-in-man study

(2013) Timothy M Miller et al.   LANCET NEUROLOGY

Drosophila melanogaster as a model organism for Alzheimer’s disease

(2013) Katja Prüßing et al.   Molecular Neurodegeneration

Normal cognition in transgenic BRI2-Aβ mice

(2013) Jungsu Kim et al.   Molecular Neurodegeneration

Meta-analysis of 74,046 individuals identifies 11 new susceptibility loci for Alzheimer's disease

(2013) Jean-Charles Lambert et al.   NATURE GENETICS

Parthanatos mediates AIMP2-activated age-dependent dopaminergic neuronal loss

(2013) Yunjong Lee et al.   NATURE NEUROSCIENCE

ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43

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

Genetics of Parkinson's Disease

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

Animal Models of Alzheimer Disease

(2013) F. M. LaFerla 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

Nonhuman Primate Models of Alzheimer-Like Cerebral Proteopathy

(2012) Eric Heuer et al.   CURRENT PHARMACEUTICAL DESIGN

Conditional Expression of Parkinson's Disease-Related Mutant  -Synuclein in the Midbrain Dopaminergic Neurons Causes Progressive Neurodegeneration and Degradation of Transcription Factor Nuclear Receptor Related 1

(2012) X. Lin et al.   JOURNAL OF NEUROSCIENCE

LRRK2 Inhibition Attenuates Microglial Inflammatory Responses

(2012) M. S. Moehle et al.   JOURNAL OF NEUROSCIENCE

Animal models of Parkinson's disease: Limits and relevance to neuroprotection studies

(2012) Erwan Bezard et al.   MOVEMENT DISORDERS

Animal models of the non-motor features of Parkinson's disease

(2012) Kimberly McDowell et al.   NEUROBIOLOGY OF DISEASE

Propagation of Tau Pathology in a Model of Early Alzheimer's Disease

(2012) Alix de Calignon et al.   NEURON

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

(2012) K. C. Luk et al.   SCIENCE

Mechanisms of Protein Seeding in Neurodegenerative Diseases

(2012) Lary C. Walker et al.   JAMA Neurology

Recent Advances in the Genetics of Parkinson's Disease

(2011) Ian Martin et al.   Annual Review of Genomics and Human Genetics

Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments

(2011) Vivek Swarup et al.   BRAIN

PARIS (ZNF746) Repression of PGC-1α Contributes to Neurodegeneration in Parkinson's Disease

(2011) Joo-Ho Shin et al.   CELL

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

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

Anti-Aβ Therapeutics in Alzheimer's Disease: The Need for a Paradigm Shift

(2011) Todd E. Golde et al.   NEURON

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

Loss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesis

(2010) Brian C. Kraemer et al.   ACTA NEUROPATHOLOGICA

Guidelines for preclinical animal research in ALS/MND: A consensus meeting

(2010) Albert C. Ludolph et al.   Amyotrophic Lateral Sclerosis

Alzheimer’s Prevention Initiative: a proposal to evaluate presymptomatic treatments as quickly as possible

(2010) Eric M Reiman et al.   Biomarkers in Medicine

TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration

(2010) C. Lagier-Tourenne et al.   HUMAN MOLECULAR GENETICS

Inhibitors of leucine-rich repeat kinase-2 protect against models of Parkinson's disease

(2010) Byoung Dae Lee et al.   NATURE MEDICINE

Prion-like transmission of protein aggregates in neurodegenerative diseases

(2010) Patrik Brundin et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

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

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

Probing the Biology of Alzheimer's Disease in Mice

(2010) Karen H. Ashe et al.   NEURON

Genetic Animal Models of Parkinson's Disease

(2010) Ted M. Dawson et al.   NEURON

Peripherally Applied A -Containing Inoculates Induce Cerebral  -Amyloidosis

(2010) Y. S. Eisele et al.   SCIENCE

TDP-43 Is a Developmentally Regulated Protein Essential for Early Embryonic Development

(2009) Chantelle F. Sephton 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

Absence of nigral degeneration in aged parkin/DJ-1/PINK1 triple knockout mice

(2009) Tohru Kitada et al.   JOURNAL OF NEUROCHEMISTRY

Bacterial Artificial Chromosome Transgenic Mice Expressing a Truncated Mutant Parkin Exhibit Age-Dependent Hypokinetic Motor Deficits, Dopaminergic Neuron Degeneration, and Accumulation of Proteinase K-Resistant  -Synuclein

(2009) X.-H. Lu et al.   JOURNAL OF NEUROSCIENCE

Design, power, and interpretation of studies in the standard murine model of ALS

(2008) Sean Scott et al.   Amyotrophic Lateral Sclerosis

Protein folding diseases and neurodegeneration: Lessons learned from yeast

(2008) Joris Winderickx et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Treatment with edaravone, initiated at symptom onset, slows motor decline and decreases SOD1 deposition in ALS mice

(2008) Hidefumi Ito et al.   EXPERIMENTAL NEUROLOGY