Motor Neuron Susceptibility in ALS/FTD

Mutant TDP-43 Causes Early-Stage Dose-Dependent Motor Neuron Degeneration in a TARDBP Knockin Mouse Model of ALS

(2019) Sarah Y. Ebstein et al.   Cell Reports

Disrupted neuronal trafficking in amyotrophic lateral sclerosis

(2019) Katja Burk et al.   ACTA NEUROPATHOLOGICA

Excitatory and Inhibitory Neuronal Circuits in the Spinal Cord and Their Role in the Control of Motor Neuron Function and Degeneration

(2018) Uri Nimrod Ramírez-Jarquín et al.   ACS Chemical Neuroscience

Energy metabolism in ALS: an underappreciated opportunity?

(2018) Tijs Vandoorne et al.   ACTA NEUROPATHOLOGICA

α-Motor neurons are spared from aging while their synaptic inputs degenerate in monkeys and mice

(2018) Nicholas Maxwell et al.   AGING CELL

The snowball effect of RNA binding protein dysfunction in amyotrophic lateral sclerosis

(2018) Pietro Fratta et al.   BRAIN

TDP-43 regulates the alternative splicing of hnRNP A1 to yield an aggregation-prone variant in amyotrophic lateral sclerosis

(2018) Jade-Emmanuelle Deshaies et al.   BRAIN

Driven to decay: Excitability and synaptic abnormalities in amyotrophic lateral sclerosis

(2018) Matthew J. Fogarty   BRAIN RESEARCH BULLETIN

Astrocytes expressing ALS-linked mutant FUS induce motor neuron death through release of tumor necrosis factor-alpha

(2018) Azadeh Kia et al.   GLIA

ERp57 is protective against mutant SOD1-induced cellular pathology in amyotrophic lateral sclerosis

(2018) Sonam Parakh et al.   HUMAN MOLECULAR GENETICS

The vulnerability of spinal motoneurons and soma size plasticity in a mouse model of amyotrophic lateral sclerosis

(2018) S. Shekar Dukkipati et al.   JOURNAL OF PHYSIOLOGY-LONDON

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

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

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

DNA plasticity and damage in amyotrophic lateral sclerosis

(2018) Alexandra Kretz et al.   Neural Regeneration Research

Normal aging induces A1-like astrocyte reactivity

(2018) Laura E. Clarke et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ALS Genes in the Genomic Era and their Implications for FTD

(2018) Hung Phuoc Nguyen et al.   TRENDS IN GENETICS

ALS-related human cortical and motor neurons survival is differentially affected by Sema3A

(2018) Anastasya Birger et al.   Cell Death & Disease

Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation

(2018) Marcel Naumann et al.   Nature Communications

Abnormal RNA stability in amyotrophic lateral sclerosis

(2018) E. M. Tank et al.   Nature Communications

Recurrent excitation between motoneurones propagates across segments and is purely glutamatergic

(2018) Gardave S. Bhumbra et al.   PLOS BIOLOGY

Selective vulnerability in neurodegenerative diseases

(2018) Hongjun Fu et al.   NATURE NEUROSCIENCE

C9orf72-mediated ALS and FTD: multiple pathways to disease

(2018) Rubika Balendra et al.   Nature Reviews Neurology

The Emerging Role of DNA Damage in the Pathogenesis of the C9orf72 Repeat Expansion in Amyotrophic Lateral Sclerosis

(2018) Anna Konopka et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Meta-analysis of Genetic Modifiers Reveals Candidate Dysregulated Pathways in Amyotrophic Lateral Sclerosis

(2018) Katherine S. Yanagi et al.   NEUROSCIENCE

Astrocyte Heterogeneity in the Adult Central Nervous System

(2018) Sean J. Miller   Frontiers in Cellular Neuroscience

Patient-derived frontotemporal lobar degeneration brain extracts induce formation and spreading of TDP-43 pathology in vivo

(2018) Sílvia Porta et al.   Nature Communications

Aggregated SOD1 causes selective death of cultured human motor neurons

(2018) Chen Benkler et al.   Scientific Reports

Reduction of matrix metalloproteinase 9 (MMP-9) protects motor neurons from TDP-43-triggered death in rNLS8 mice

(2018) Krista J. Spiller et al.   NEUROBIOLOGY OF DISEASE

Ageing as a risk factor for ALS/FTD

(2017) Teresa Niccoli et al.   HUMAN MOLECULAR GENETICS

Mitochondrial abnormalities and disruption of the neuromuscular junction precede the clinical phenotype and motor neuron loss in hFUSWT transgenic mice

(2017) Eva So et al.   HUMAN MOLECULAR GENETICS

The DNA damage response (DDR) is induced by the C9orf72 repeat expansion in amyotrophic lateral sclerosis

(2017) Manal A. Farg et al.   HUMAN MOLECULAR GENETICS

Microglia and C9orf72 in neuroinflammation and ALS and frontotemporal dementia

(2017) Deepti Lall et al.   JOURNAL OF CLINICAL INVESTIGATION

ALS genetic epidemiology ‘How simplex is the genetic epidemiology of ALS?’

(2017) Jan H Veldink   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Cortical influences drive amyotrophic lateral sclerosis

(2017) Andrew Eisen et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Kinnier Wilson’s puzzling features of amyotrophic lateral sclerosis

(2017) Martin R Turner et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Genetic epidemiology of amyotrophic lateral sclerosis: a systematic review and meta-analysis

(2017) Zhang-Yu Zou et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Neurotoxic reactive astrocytes are induced by activated microglia

(2017) Shane A. Liddelow et al.   NATURE

C9orf72 expansion disrupts ATM-mediated chromosomal break repair

(2017) Callum Walker et al.   NATURE NEUROSCIENCE

Motor cortex — to act or not to act?

(2017) Christian Laut Ebbesen et al.   NATURE REVIEWS NEUROSCIENCE

Investigating the role of ALS genes CHCHD10 and TUBA4A in Belgian FTD-ALS spectrum patients

(2017) Federica Perrone et al.   NEUROBIOLOGY OF AGING

Novel UBQLN2 mutations linked to amyotrophic lateral sclerosis and atypical hereditary spastic paraplegia phenotype through defective HSP70-mediated proteolysis

(2017) Elisa Teyssou et al.   NEUROBIOLOGY OF AGING

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

(2017) Kurt J. De Vos et al.   NEUROBIOLOGY OF DISEASE

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

(2017) Hong Joo Kim et al.   NEURON

The ER proteostasis network in ALS: Determining the differential motoneuron vulnerability

(2017) Pablo Rozas et al.   NEUROSCIENCE LETTERS

DCTN1 gene analysis in Chinese patients with sporadic amyotrophic lateral sclerosis

(2017) Xiangyi Liu et al.   PLoS One

DNA strand breaks and TDP-43 mislocation are absent in the murine hSOD1G93A model of amyotrophic lateral sclerosis in vivo and in vitro

(2017) Diane Penndorf et al.   PLoS One

Transfer of pathogenic and nonpathogenic cytosolic proteins between spinal cord motor neurons in vivo in chimeric mice

(2017) Eleanor V. Thomas et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mouse Models of C9orf72 Hexanucleotide Repeat Expansion in Amyotrophic Lateral Sclerosis/ Frontotemporal Dementia

(2017) Ranjan Batra et al.   Frontiers in Cellular Neuroscience

Progressive Motor Neuron Pathology and the Role of Astrocytes in a Human Stem Cell Model of VCP-Related ALS

(2017) Claire E. Hall et al.   Cell Reports

Patterns of symptom development in patients with motor neuron disease

(2017) Renée Walhout et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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

(2017) Hamideh Shahheydari et al.   Frontiers in Molecular Neuroscience

Structural insights into human angiogenin variants implicated in Parkinson’s disease and Amyotrophic Lateral Sclerosis

(2017) William J. Bradshaw et al.   Scientific Reports

ALS Associated Mutations in Matrin 3 Alter Protein-Protein Interactions and Impede mRNA Nuclear Export

(2017) Ashley Boehringer et al.   Scientific Reports

NEK1mutations in familial amyotrophic lateral sclerosis

(2016) David Brenner et al.   BRAIN

Proteostasis impairment in ALS

(2016) Céline Ruegsegger et al.   BRAIN RESEARCH

Neuronal energy consumption: biophysics, efficiency and evolution

(2016) Jeremy E Niven   CURRENT OPINION IN NEUROBIOLOGY

Semaphorin 3A is a retrograde cell death signal in developing sympathetic neurons

(2016) Amanda B. Wehner et al.   DEVELOPMENT

A new mode of mitochondrial transport and polarized sorting regulated by Dynein, Milton and Miro

(2016) Anna Melkov et al.   DEVELOPMENT

ALS‐linked protein disulfide isomerase variants cause motor dysfunction

(2016) Ute Woehlbier et al.   EMBO JOURNAL

ER strikes again: Proteostasis Dysfunction In ALS

(2016) Niran Maharjan et al.   EMBO JOURNAL

TDP‐43 loss of function inhibits endosomal trafficking and alters trophic signaling in neurons

(2016) Benjamin M Schwenk et al.   EMBO JOURNAL

The Axon Guidance Protein Semaphorin 3A Is Increased in the Motor Cortex of Patients With Amyotrophic Lateral Sclerosis

(2016) Sonja Körner et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Selective Motor Neuron Resistance and Recovery in a New Inducible Mouse Model of TDP-43 Proteinopathy

(2016) K. J. Spiller et al.   JOURNAL OF NEUROSCIENCE

IL-10 Controls Early Microglial Phenotypes and Disease Onset in ALS Caused by Misfolded Superoxide Dismutase 1

(2016) M. Gravel et al.   JOURNAL OF NEUROSCIENCE

Physiology of the fasciculation potentials in amyotrophic lateral sclerosis: which motor units fasciculate?

(2016) Mamede de Carvalho et al.   Journal of Physiological Sciences

New perspectives on amyotrophic lateral sclerosis: the role of glial cells at the neuromuscular junction

(2016) Danielle Arbour et al.   JOURNAL OF PHYSIOLOGY-LONDON

The frontotemporal dementia-motor neuron disease continuum

(2016) James R Burrell et al.   LANCET

The Mitochondrial Basis of Aging

(2016) Nuo Sun et al.   MOLECULAR CELL

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

Genome-wide association analyses identify new risk variants and the genetic architecture of amyotrophic lateral sclerosis

(2016) Wouter van Rheenen et al.   NATURE GENETICS

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

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

Decoding the organization of spinal circuits that control locomotion

(2016) Ole Kiehn   NATURE REVIEWS NEUROSCIENCE

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

(2016) Phillip Smethurst et al.   NEUROBIOLOGY OF DISEASE

Gamma motor neurons survive and exacerbate alpha motor neuron degeneration in ALS

(2016) Melanie Lalancette-Hebert et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Two familial ALS proteins function in prevention/repair of transcription-associated DNA damage

(2016) Sarah J. Hill et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function

(2016) Aarti Sharma et al.   Nature Communications

CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia

(2016) Kelly L. Williams et al.   Nature Communications

Differential neuronal vulnerability identifies IGF-2 as a protective factor in ALS

(2016) Ilary Allodi et al.   Scientific Reports

Cell-to-Cell Transmission of Dipeptide Repeat Proteins Linked to C9orf72 -ALS/FTD

(2016) Thomas Westergard et al.   Cell Reports

The selective anatomical vulnerability of ALS: ‘disease-defining’ and ‘disease-defying’ brain regions

(2016) Peter Bede et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Progression of motor neuron disease is accelerated and the ability to recover is compromised with advanced age in rNLS8 mice

(2016) Krista J. Spiller et al.   Acta Neuropathologica Communications

The NEK1 interactor, C21ORF2, is required for efficient DNA damage repair

(2015) Xiao Fang et al.   ACTA BIOCHIMICA ET BIOPHYSICA SINICA

Aberrant association of misfolded SOD1 with Na+/K+ATPase-α3 impairs its activity and contributes to motor neuron vulnerability in ALS

(2015) Céline Ruegsegger et al.   ACTA NEUROPATHOLOGICA

Rab1-dependent ER–Golgi transport dysfunction is a common pathogenic mechanism in SOD1, TDP-43 and FUS-associated ALS

(2015) Kai Y. Soo 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

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

Prion-like propagation of mutant SOD1 misfolding and motor neuron disease spread along neuroanatomical pathways

(2015) Jacob I. Ayers et al.   ACTA NEUROPATHOLOGICA

Whole-genome sequencing reveals important role for TBK1 and OPTN mutations in frontotemporal lobar degeneration without motor neuron disease

(2015) Cyril Pottier et al.   ACTA NEUROPATHOLOGICA

Analysis of theCHCHD10gene in patients with frontotemporal dementia and amyotrophic lateral sclerosis from Spain

(2015) Oriol Dols-Icardo et al.   BRAIN

Mutation analysis ofCHCHD10in different neurodegenerative diseases

(2015) Ming Zhang et al.   BRAIN

Cortical hyperexcitability precedes lower motor neuron dysfunction in ALS

(2015) Parvathi Menon et al.   CLINICAL NEUROPHYSIOLOGY

TDP-43 as a Modulator of Synaptic Plasticity in a Mouse Model of Spinal Motoneuron Degeneration

(2015) Rosario Gulino et al.   CNS & Neurological Disorders-Drug Targets

TDP-43 is intercellularly transmitted across axon terminals

(2015) Marisa S. Feiler et al.   JOURNAL OF CELL BIOLOGY

Cross-disease comparison of amyotrophic lateral sclerosis and spinal muscular atrophy reveals conservation of selective vulnerability but differential neuromuscular junction pathology

(2015) Laura H. Comley et al.   JOURNAL OF COMPARATIVE NEUROLOGY

Homeostatic Dysregulation in Membrane Properties of Masticatory Motoneurons Compared with Oculomotor Neurons in a Mouse Model for Amyotrophic Lateral Sclerosis

(2015) S. Venugopal et al.   JOURNAL OF NEUROSCIENCE

Aging, the Central Nervous System, and Mobility in Older Adults: Neural Mechanisms of Mobility Impairment

(2015) Farzaneh A. Sorond et al.   JOURNALS OF GERONTOLOGY SERIES A-BIOLOGICAL SCIENCES AND MEDICAL SCIENCES

Cellular Signature of SIL1 Depletion: Disease Pathogenesis due to Alterations in Protein Composition Beyond the ER Machinery

(2015) Andreas Roos et al.   MOLECULAR NEUROBIOLOGY

Autonomic system and amyotrophic lateral sclerosis

(2015) Ezequiel A. Piccione et al.   MUSCLE & NERVE

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport

(2015) Ke Zhang et al.   NATURE

An siRNA-based functional genomics screen for the identification of regulators of ciliogenesis and ciliopathy genes

(2015) Gabrielle Wheway et al.   NATURE CELL BIOLOGY

Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS

(2015) Mercedes Prudencio et al.   NATURE NEUROSCIENCE

Marinesco-Sjögren syndrome protein SIL1 regulates motor neuron subtype-selective ER stress in ALS

(2015) Audrey Filézac de L'Etang et al.   NATURE NEUROSCIENCE

Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia

(2015) Axel Freischmidt et al.   NATURE NEUROSCIENCE

Astrocytes show reduced support of motor neurons with aging that is accelerated in a rodent model of ALS

(2015) Melanie M. Das et al.   NEUROBIOLOGY OF AGING

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

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

Motor neurons with differential vulnerability to degeneration show distinct protein signatures in health and ALS

(2015) L. Comley et al.   NEUROSCIENCE

TDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets

(2015) Emma L. Scotter et al.   Neurotherapeutics

Linking RNA Dysfunction and Neurodegeneration in Amyotrophic Lateral Sclerosis

(2015) Sami J. Barmada   Neurotherapeutics

Translational profiling identifies a cascade of damage initiated in motor neurons and spreading to glia in mutant SOD1-mediated ALS

(2015) Shuying Sun et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Exome sequencing in amyotrophic lateral sclerosis identifies risk genes and pathways

(2015) E. T. Cirulli et al.   SCIENCE

From nucleation to widespread propagation: A prion-like concept for ALS

(2015) Zuzanna Maniecka et al.   VIRUS RESEARCH

Activation of the endoplasmic reticulum stress response in skeletal muscle of G93A*SOD1 amyotrophic lateral sclerosis mice

(2015) Dapeng Chen et al.   Frontiers in Cellular Neuroscience

Human iPSC-derived motoneurons harbouring TARDBP or C9ORF72 ALS mutations are dysfunctional despite maintaining viability

(2015) Anna-Claire Devlin et al.   Nature Communications

Exposure to ALS-FTD-CSF generates TDP-43 aggregates in glioblastoma cells through exosomes and TNTs-like structure

(2015) Xuebing Ding et al.   Oncotarget

Astrocyte-Derived TGF-β1 Accelerates Disease Progression in ALS Mice by Interfering with the Neuroprotective Functions of Microglia and T Cells

(2015) Fumito Endo et al.   Cell Reports

Eye-tracking in amyotrophic lateral sclerosis: A longitudinal study of saccadic and cognitive tasks

(2015) Malcolm Proudfoot et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Prion-like Mechanism in Amyotrophic Lateral Sclerosis: are Protein Aggregates the Key?

(2015) Shynrye Lee et al.   Experimental Neurobiology

The C9ORF72 expansion mutation: gene structure, phenotypic and diagnostic issues

(2014) Ione O. C. Woollacott et al.   ACTA NEUROPATHOLOGICA

Experimental transmissibility of mutant SOD1 motor neuron disease

(2014) Jacob I. Ayers et al.   ACTA NEUROPATHOLOGICA

Phenotype of matrin-3-related distal myopathy in 16 German patients

(2014) Tobias J. Müller et al.   ANNALS OF NEUROLOGY

Mutations in the CHCHD10 gene are a common cause of familial amyotrophic lateral sclerosis

(2014) Janel O. Johnson et al.   BRAIN

A mitochondrial origin for frontotemporal dementia and amyotrophic lateral sclerosis through CHCHD10 involvement

(2014) Sylvie Bannwarth et al.   BRAIN

Pathways Disrupted in Human ALS Motor Neurons Identified through Genetic Correction of Mutant SOD1

(2014) Evangelos Kiskinis et al.   Cell Stem Cell

Mechanisms of astrocyte development and their contributions to neurodevelopmental disorders

(2014) Steven A Sloan et al.   CURRENT OPINION IN NEUROBIOLOGY

C9ORF72, implicated in amytrophic lateral sclerosis and frontotemporal dementia, regulates endosomal trafficking

(2014) Manal A. Farg et al.   HUMAN MOLECULAR GENETICS

Profiling the genes affected by pathogenic TDP-43 in astrocytes

(2014) Cao Huang et al.   JOURNAL OF NEUROCHEMISTRY

Semaphorin 3A Signaling Through Neuropilin-1 Is an Early Trigger for Distal Axonopathy in the SOD1G93AMouse Model of Amyotrophic Lateral Sclerosis

(2014) Kalina Venkova et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Delayed Disease Onset and Extended Survival in the SOD1G93A Rat Model of Amyotrophic Lateral Sclerosis after Suppression of Mutant SOD1 in the Motor Cortex

(2014) G. M. Thomsen et al.   JOURNAL OF NEUROSCIENCE

Role of Lamin B1 in Chromatin Instability

(2014) Veronika Butin-Israeli et al.   MOLECULAR AND CELLULAR BIOLOGY

Preclinical research: Make mouse studies work

(2014) Steve Perrin   NATURE

Astrocyte-encoded positional cues maintain sensorimotor circuit integrity

(2014) Anna V. Molofsky et al.   NATURE

Microglial priming in neurodegenerative disease

(2014) V. Hugh Perry et al.   Nature Reviews Neurology

The phenotypic variability of amyotrophic lateral sclerosis

(2014) Bart Swinnen et al.   Nature Reviews Neurology

Mutations in UBQLN2 and SIGMAR1 genes are rare in Korean patients with amyotrophic lateral sclerosis

(2014) Hee-Jung Kim et al.   NEUROBIOLOGY OF AGING

A Computational Model of Motor Neuron Degeneration

(2014) Gwendal Le Masson et al.   NEURON

Neuronal Matrix Metalloproteinase-9 Is a Determinant of Selective Neurodegeneration

(2014) Artem Kaplan et al.   NEURON

DNA Damage and Its Links to Neurodegeneration

(2014) Ram Madabhushi et al.   NEURON

Exome-wide Rare Variant Analysis Identifies TUBA4A Mutations Associated with Familial ALS

(2014) Bradley N. Smith et al.   NEURON

Late-Onset of Spinal Neurodegeneration in Knock-In Mice Expressing a Mutant BiP

(2014) Hisayo Jin et al.   PLoS One

Selective degeneration of a physiological subtype of spinal motor neuron in mice with SOD1-linked ALS

(2014) Muhamed Hadzipasic et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Astrocytes expressing mutant SOD1 and TDP43 trigger motoneuron death that is mediated via sodium channels and nitroxidative stress

(2014) Fabiola Rojas et al.   Frontiers in Cellular Neuroscience

Motor neurons and the generation of spinal motor neuron diversity

(2014) Nicolas Stifani   Frontiers in Cellular Neuroscience

Superoxide dismutase 1 acts as a nuclear transcription factor to regulate oxidative stress resistance

(2014) Chi Kwan Tsang et al.   Nature Communications

Intrinsic Membrane Hyperexcitability of Amyotrophic Lateral Sclerosis Patient-Derived Motor Neurons

(2014) Brian J. Wainger et al.   Cell Reports

Early intrinsic hyperexcitability does not contribute to motoneuron degeneration in amyotrophic lateral sclerosis

(2014) Félix Leroy et al.   eLife

Frontotemporal Dementia Associated With theC9ORF72Mutation

(2014) Emma Devenney et al.   JAMA Neurology

Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations

(2013) Ian R. Mackenzie et al.   ACTA NEUROPATHOLOGICA

Large C9orf72 Hexanucleotide Repeat Expansions Are Seen in Multiple Neurodegenerative Syndromes and Are More Frequent Than Expected in the UK Population

(2013) Jon Beck et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Stages of pTDP-43 pathology in amyotrophic lateral sclerosis

(2013) Johannes Brettschneider et al.   ANNALS OF NEUROLOGY

Genome Instability and Aging

(2013) Jan Vijg et al.   Annual Review of Physiology

Oligodendrocyte dysfunction in the pathogenesis of amyotrophic lateral sclerosis

(2013) Thomas Philips et al.   BRAIN

Loss of TDP-43 causes age-dependent progressive motor neuron degeneration

(2013) Yohei Iguchi et al.   BRAIN

Is SOD1 loss of function involved in amyotrophic lateral sclerosis?

(2013) Rachele A. Saccon et al.   BRAIN

Extracellular wildtype and mutant SOD1 induces ER–Golgi pathology characteristic of amyotrophic lateral sclerosis in neuronal cells

(2013) Vinod Sundaramoorthy et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

The multifaceted role of glial cells in amyotrophic lateral sclerosis

(2013) Chiara F. Valori et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

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

Overexpression of human mutated G93A SOD1 changes dynamics of the ER mitochondria calcium cycle specifically in mouse embryonic motor neurons

(2013) Janin Lautenschläger et al.   EXPERIMENTAL NEUROLOGY

Investigating the contribution of VAPB/ALS8 loss of function in amyotrophic lateral sclerosis

(2013) Edor Kabashi et al.   HUMAN MOLECULAR GENETICS

The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons

(2013) Wenzhang Wang et al.   HUMAN MOLECULAR GENETICS

Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models

(2013) Jordi Magrané et al.   HUMAN MOLECULAR GENETICS

TARDBPandFUSMutations Associated with Amyotrophic Lateral Sclerosis: Summary and Update

(2013) Serena Lattante et al.   HUMAN MUTATION

Na+/K+ ATPase  1 and  3 Isoforms Are Differentially Expressed in  - and  -Motoneurons

(2013) I. J. Edwards et al.   JOURNAL OF NEUROSCIENCE

Mitochondria, motor neurons and aging

(2013) M.L. García et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Association between repeat sizes and clinical and pathological characteristics in carriers of C9ORF72 repeat expansions (Xpansize-72): a cross-sectional cohort study

(2013) Marka van Blitterswijk et al.   LANCET NEUROLOGY

Potential Effect of S-Nitrosylated Protein Disulfide Isomerase on Mutant SOD1 Aggregation and Neuronal Cell Death in Amyotrophic Lateral Sclerosis

(2013) Gye Sun Jeon et al.   MOLECULAR NEUROBIOLOGY

Tools and talk: An evolutionary perspective on the functional deficits associated with amyotrophic lateral sclerosis

(2013) Andrew Eisen et al.   MUSCLE & NERVE

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

(2013) Hong Joo Kim et al.   NATURE

Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis

(2013) Shin H Kang et al.   NATURE NEUROSCIENCE

State of play in amyotrophic lateral sclerosis genetics

(2013) Alan E Renton et al.   NATURE NEUROSCIENCE

Amyotrophic lateral sclerosis—a model of corticofugal axonal spread

(2013) Heiko Braak et al.   Nature Reviews Neurology

Explorative genetic study of UBQLN2 and PFN1 in an extended Flanders-Belgian cohort of frontotemporal lobar degeneration patients

(2013) Lubina Dillen et al.   NEUROBIOLOGY OF AGING

Lack of C9ORF72 coding mutations supports a gain of function for repeat expansions in amyotrophic lateral sclerosis

(2013) Matthew B. Harms et al.   NEUROBIOLOGY OF AGING

Neuroprotection through Excitability and mTOR Required in ALS Motoneurons to Delay Disease and Extend Survival

(2013) Smita Saxena et al.   NEURON

Spinal inhibitory circuits and their role in motor neuron degeneration

(2013) Uri Nimrod Ramírez-Jarquín et al.   NEUROPHARMACOLOGY

De Novo Mutations in Ataxin-2 Gene and ALS Risk

(2013) José Miguel Laffita-Mesa et al.   PLoS One

ALS-Associated TDP-43 Induces Endoplasmic Reticulum Stress, Which Drives Cytoplasmic TDP-43 Accumulation and Stress Granule Formation

(2013) Adam K. Walker et al.   PLoS One

Cortical energy demands of signaling and nonsignaling components in brain are conserved across mammalian species and activity levels

(2013) Fahmeed Hyder et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Cell and neuron densities in the primary motor cortex of primates

(2013) Nicole A. Young et al.   Frontiers in Neural Circuits

Heterogeneous neural coding of corrective movements in motor cortex

(2013) Adam S. Dickey et al.   Frontiers in Neural Circuits

In vivo optogenetic tracing of functional corticocortical connections between motor forelimb areas

(2013) Riichiro Hira et al.   Frontiers in Neural Circuits

Mapping the spatio-temporal structure of motor cortical LFP and spiking activities during reach-to-grasp movements

(2013) Alexa Riehle et al.   Frontiers in Neural Circuits

Prion-like Properties of Pathological TDP-43 Aggregates from Diseased Brains

(2013) Takashi Nonaka et al.   Cell Reports

A Neurodegeneration-Specific Gene-Expression Signature of Acutely Isolated Microglia from an Amyotrophic Lateral Sclerosis Mouse Model

(2013) Isaac M. Chiu et al.   Cell Reports

Eye tracking communication devices in amyotrophic lateral sclerosis: Impact on disability and quality of life

(2013) Marco Caligari et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

SQSTM1Mutations in French Patients With Frontotemporal Dementia or Frontotemporal Dementia With Amyotrophic Lateral Sclerosis

(2013) Isabelle Le Ber   JAMA Neurology

Characterization of early pathogenesis in the SOD1G93Amouse model of ALS: part II, results and discussion

(2013) Sharon Vinsant et al.   Brain and Behavior

Amyotrophic lateral sclerosis (ALS)-associated VAPB-P56S inclusions represent an ER quality control compartment

(2013) Marijn Kuijpers et al.   Acta Neuropathologica Communications

Unravelling the enigma of selective vulnerability in neurodegeneration: motor neurons resistant to degeneration in ALS show distinct gene expression characteristics and decreased susceptibility to excitotoxicity

(2012) Alice Brockington et al.   ACTA NEUROPATHOLOGICA

Clinical and pathological features of familial frontotemporal dementia caused by C9ORF72 mutation on chromosome 9p

(2012) G.-Y. R. Hsiung et al.   BRAIN

Frontotemporal dementia with the C9ORF72 hexanucleotide repeat expansion: clinical, neuroanatomical and neuropathological features

(2012) C. J. Mahoney et al.   BRAIN

Transmissible Proteins: Expanding the Prion Heresy

(2012) Claudio Soto   CELL

Aging and muscle

(2012) Todd M. Manini et al.   CURRENT OPINION IN CLINICAL NUTRITION AND METABOLIC CARE

Regional spread pattern predicts survival in patients with sporadic amyotrophic lateral sclerosis

(2012) G. M. Gargiulo-Monachelli et al.   EUROPEAN JOURNAL OF NEUROLOGY

Transformation from a neuroprotective to a neurotoxic microglial phenotype in a mouse model of ALS

(2012) Bing Liao et al.   EXPERIMENTAL NEUROLOGY

Entorhinal cortical neurons are the primary targets of FUS mislocalization and ubiquitin aggregation in FUS transgenic rats

(2012) Cao Huang et al.   HUMAN MOLECULAR GENETICS

Evidence for an oligogenic basis of amyotrophic lateral sclerosis

(2012) Marka van Blitterswijk et al.   HUMAN MOLECULAR GENETICS

A Pan-European Study of theC9orf72Repeat Associated with FTLD: Geographic Prevalence, Genomic Instability, and Intermediate Repeats

(2012) Julie van der Zee et al.   HUMAN MUTATION

Cognitive decline and reduced survival inC9orf72expansion frontotemporal degeneration and amyotrophic lateral sclerosis

(2012) David J Irwin et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Reduced Calreticulin Levels Link Endoplasmic Reticulum Stress and Fas-Triggered Cell Death in Motoneurons Vulnerable to ALS

(2012) N. Bernard-Marissal et al.   JOURNAL OF NEUROSCIENCE

Wnt7A Identifies Embryonic  -Motor Neurons and Reveals Early Postnatal Dependence of  -Motor Neurons on a Muscle Spindle-Derived Signal

(2012) S. Ashrafi et al.   JOURNAL OF NEUROSCIENCE

Glutamate modulates the firing rate in oculomotor nucleus motoneurons as a function of the recruitment threshold current

(2012) Julio Torres-Torrelo et al.   JOURNAL OF PHYSIOLOGY-LONDON

Expression of postsynaptic Ca2+-activated K+(SK) channels at C-bouton synapses in mammalian lumbar α-motoneurons

(2012) Adam S. Deardorff et al.   JOURNAL OF PHYSIOLOGY-LONDON

Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study

(2012) Elisa Majounie et al.   LANCET NEUROLOGY

Sensorimotor function is modulated by the serotonin receptor 1d, a novel marker for gamma motor neurons

(2012) Anders Enjin et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis

(2012) Chi-Hong Wu et al.   NATURE

EPHA4 is a disease modifier of amyotrophic lateral sclerosis in animal models and in humans

(2012) Annelies Van Hoecke et al.   NATURE MEDICINE

Mutant FUS induces endoplasmic reticulum stress in amyotrophic lateral sclerosis and interacts with protein disulfide-isomerase

(2012) Manal A. Farg et al.   NEUROBIOLOGY OF AGING

Length of normal alleles of C9ORF72 GGGGCC repeat do not influence disease phenotype

(2012) Nicola J. Rutherford et al.   NEUROBIOLOGY OF AGING

Screening in ALS and FTD patients reveals 3 novel UBQLN2 mutations outside the PXX domain and a pure FTD phenotype

(2012) Matthis Synofzik et al.   NEUROBIOLOGY OF AGING

UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis

(2012) Kelly L. Williams et al.   NEUROBIOLOGY OF AGING

Selective knockdown of mutant SOD1 in Schwann cells ameliorates disease in G85R mutant SOD1 transgenic mice

(2012) Lijun Wang et al.   NEUROBIOLOGY OF DISEASE

SQSTM1 mutations in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

(2012) E. Rubino et al.   NEUROLOGY

Synaptic Energy Use and Supply

(2012) Julia J. Harris et al.   NEURON

What is dynapenia?

(2012) Brian C. Clark et al.   NUTRITION

Shared Resistance to Aging and ALS in Neuromuscular Junctions of Specific Muscles

(2012) Gregorio Valdez et al.   PLoS One

A role for calpain-dependent cleavage of TDP-43 in amyotrophic lateral sclerosis pathology

(2012) Takenari Yamashita et al.   Nature Communications

Pathological heterogeneity in amyotrophic lateral sclerosis with FUS mutations: two distinct patterns correlating with disease severity and mutation

(2011) Ian R. A. Mackenzie et al.   ACTA NEUROPATHOLOGICA

Clinical and neuropathologic heterogeneity of c9FTD/ALS associated with hexanucleotide repeat expansion in C9ORF72

(2011) Melissa E. Murray et al.   ACTA NEUROPATHOLOGICA

Mutational analysis reveals the FUS homolog TAF15 as a candidate gene for familial amyotrophic lateral sclerosis

(2011) N. Ticozzi et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART B-NEUROPSYCHIATRIC GENETICS

SQSTM1 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2011) Faisal Fecto   ARCHIVES OF NEUROLOGY

Blunting type 1 insulin-like growth factor receptor expression exacerbates neuronal apoptosis following hypoxic/ischemic injury

(2011) Wen Liu et al.   BMC NEUROSCIENCE

Global gene expression profiling of somatic motor neuron populations with different vulnerability identify molecules and pathways of degeneration and protection

(2011) Eva Hedlund et al.   BRAIN

Endogenous regulatory T lymphocytes ameliorate amyotrophic lateral sclerosis in mice and correlate with disease progression in patients with amyotrophic lateral sclerosis

(2011) David R. Beers et al.   BRAIN

Morphological changes in nerve cells during normal aging

(2011) Ennio Pannese   Brain Structure & Function

The anatomy of cognitive impairment in amyotrophic lateral sclerosis: More than frontal lobe dysfunction

(2011) Stella Tsermentseli et al.   CORTEX

Ataxin-2 intermediate-length polyglutamine expansions in European ALS patients

(2011) T. Lee et al.   HUMAN MOLECULAR GENETICS

Ataxin-2 repeat-length variation and neurodegeneration

(2011) O. A. Ross et al.   HUMAN MOLECULAR GENETICS

Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor κB–mediated pathogenic pathways

(2011) Vivek Swarup et al.   JOURNAL OF EXPERIMENTAL MEDICINE

The Frontotemporal Syndromes of ALS. Clinicopathological Correlates

(2011) Michael Joseph Strong et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

Onset and spreading patterns of lower motor neuron involvements predict survival in sporadic amyotrophic lateral sclerosis

(2011) C. Fujimura-Kiyono et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Spinal Inhibitory Interneuron Pathology Follows Motor Neuron Degeneration Independent of Glial Mutant Superoxide Dismutase 1 Expression in SOD1-ALS Mice

(2011) Mehdi Hossaini et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Osteopontin is an alpha motor neuron marker in the mouse spinal cord

(2011) Hidemi Misawa et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Pitx2 regulates myosin heavy chain isoform expression and multi-innervation in extraocular muscle

(2011) Yuefang Zhou et al.   JOURNAL OF PHYSIOLOGY-LONDON

Amyotrophic lateral sclerosis

(2011) Matthew C Kiernan et al.   LANCET

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

(2011) Han-Xiang Deng et al.   NATURE

Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43

(2011) Magdalini Polymenidou et al.   NATURE NEUROSCIENCE

UNC13A is a modifier of survival in amyotrophic lateral sclerosis

(2011) Frank P. Diekstra et al.   NEUROBIOLOGY OF AGING

Profound downregulation of the RNA editing enzyme ADAR2 in ALS spinal motor neurons

(2011) Takuto Hideyama et al.   NEUROBIOLOGY OF DISEASE

Novel p.Ile151Val mutation in VCP in a patient of African American descent with sporadic ALS

(2011) M. DeJesus-Hernandez et al.   NEUROLOGY

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

Misfolded SOD1 Associated with Motor Neuron Mitochondria Alters Mitochondrial Shape and Distribution Prior to Clinical Onset

(2011) Christine Vande Velde et al.   PLoS One

Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivo

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

Intermolecular transmission of superoxide dismutase 1 misfolding in living cells

(2011) L. I. Grad et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells

(2011) Christian Münch et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Unfolded Protein Response: From Stress Pathway to Homeostatic Regulation

(2011) P. Walter et al.   SCIENCE

The endoplasmic reticulum and protein trafficking in dendrites and axons

(2011) Omar A. Ramírez et al.   TRENDS IN CELL BIOLOGY

TDP-43 pathology in sporadic ALS occurs in motor neurons lacking the RNA editing enzyme ADAR2

(2010) Hitoshi Aizawa et al.   ACTA NEUROPATHOLOGICA

FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis

(2010) Han-Xiang Deng et al.   ANNALS OF NEUROLOGY

Motor Neuron Diversity in Development and Disease

(2010) Kevin C. Kanning et al.   Annual Review of Neuroscience

Effects of ALS-related SOD1 mutants on dynein- and KIF5-mediated retrograde and anterograde axonal transport

(2010) Ping Shi et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Retrograde influence of muscle fibers on their innervation revealed by a novel marker for slow motoneurons

(2010) J. V. Chakkalakal et al.   DEVELOPMENT

Astrocyte loss of mutant SOD1 delays ALS disease onset and progression in G85R transgenic mice

(2010) L. Wang et al.   HUMAN MOLECULAR GENETICS

Neuropilin 1 Directly Interacts with Fer Kinase to Mediate Semaphorin 3A-induced Death of Cortical Neurons

(2010) Susan X. Jiang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Identification of novel spinal cholinergic genetic subtypes disclose Chodl and Pitx2 as markers for fast motor neurons and partition cells

(2010) Anders Enjin et al.   JOURNAL OF COMPARATIVE NEUROLOGY

Eye movements in amyotrophic lateral sclerosis and its mimics: a review with illustrative cases

(2010) C. Donaghy et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Endoplasmic Reticulum Stress in Motor Neurons of the Spinal Cord in Sporadic Amyotrophic Lateral Sclerosis

(2010) Shoichi Sasaki   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Induced Loss of ADAR2 Engenders Slow Death of Motor Neurons from Q/R Site-Unedited GluR2

(2010) T. Hideyama et al.   JOURNAL OF NEUROSCIENCE

Wild-Type Human TDP-43 Expression Causes TDP-43 Phosphorylation, Mitochondrial Aggregation, Motor Deficits, and Early Mortality in Transgenic Mice

(2010) Y.-F. Xu et al.   JOURNAL OF NEUROSCIENCE

Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS

(2010) Andrew C. Elden et al.   NATURE

Mutations of optineurin in amyotrophic lateral sclerosis

(2010) Hirofumi Maruyama et al.   NATURE

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

(2010) Daryl A Bosco et al.   NATURE NEUROSCIENCE

Prion-like transmission of protein aggregates in neurodegenerative diseases

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

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

(2010) Adrian Israelson et al.   NEURON

Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS

(2010) Janel O. Johnson et al.   NEURON

Questioning on the role of D amino acid oxidase in familial amyotrophic lateral sclerosis

(2010) S. Millecamps et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Familial amyotrophic lateral sclerosis is associated with a mutation in D-amino acid oxidase

(2010) J. Mitchell et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice

(2010) X. Shan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TARDBP Mutations in Frontotemporal Lobar Degeneration: Frequency, Clinical Features, and Disease Course

(2010) B. Borroni et al.   REJUVENATION RESEARCH

Selective neuronal vulnerability to oxidative stress in the brain

(2010) Wang   Frontiers in Aging Neuroscience

Accumulation of TDP-43 and α-actin in an amyotrophic lateral sclerosis patient with the K17I ANG mutation

(2009) Danielle Seilhean et al.   ACTA NEUROPATHOLOGICA

Longitudinal study of muscle strength, quality, and adipose tissue infiltration

(2009) et al.   AMERICAN JOURNAL OF CLINICAL NUTRITION

Axonal Mitochondrial Clusters Containing Mutant SOD1 in Transgenic Models of ALS

(2009) Jose R. Sotelo-Silveira et al.   ANTIOXIDANTS & REDOX SIGNALING

Genomic and biochemical approaches in the discovery of mechanisms for selective neuronal vulnerability to oxidative stress

(2009) Xinkun Wang et al.   BMC NEUROSCIENCE

Protein disulphide isomerase protects against protein aggregation and is S-nitrosylated in amyotrophic lateral sclerosis

(2009) Adam K. Walker et al.   BRAIN

A new subtype of frontotemporal lobar degeneration with FUS pathology

(2009) M. Neumann et al.   BRAIN

Increase in presynaptic territory of C-terminals on lumbar motoneurons of G93A SOD1 mice during disease progression

(2009) A. H. Pullen et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Depletion of TDP-43 affectsDrosophila motoneuronsterminal synapsis and locomotive behavior

(2009) Fabian Feiguin et al.   FEBS LETTERS

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

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

Lamin B1 controls oxidative stress responses via Oct-1

(2009) Ashraf N. Malhas et al.   JOURNAL OF CELL BIOLOGY

ALS-linked P56S-VAPB, an aggregated loss-of-function mutant of VAPB, predisposes motor neurons to ER stress-related death by inducing aggregation of co-expressed wild-type VAPB

(2009) Hiroaki Suzuki et al.   JOURNAL OF NEUROCHEMISTRY

FUS mutations in amyotrophic lateral sclerosis: clinical, pathological, neurophysiological and genetic analysis

(2009) I. P. Blair et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Upregulation of persistent sodium conductances in familial ALS

(2009) S. Vucic et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Early Excitability Changes in Lumbar Motoneurons of Transgenic SOD1G85R and SOD1G93A-Low Mice

(2009) Arnaud Pambo-Pambo et al.   JOURNAL OF NEUROPHYSIOLOGY

Progressive Changes in Synaptic Inputs to Motoneurons in Adult Sacral Spinal Cord of a Mouse Model of Amyotrophic Lateral Sclerosis

(2009) M. Jiang et al.   JOURNAL OF NEUROSCIENCE

A Switch in Retrograde Signaling from Survival to Stress in Rapid-Onset Neurodegeneration

(2009) E. Perlson et al.   JOURNAL OF NEUROSCIENCE

A role for motoneuron subtype–selective ER stress in disease manifestations of FALS mice

(2009) Smita Saxena et al.   NATURE NEUROSCIENCE

Gamma motor neurons express distinct genetic markers at birth and require muscle spindle-derived GDNF for postnatal survival

(2009) Neil A Shneider et al.   Neural Development

Two Italian kindreds with familial amyotrophic lateral sclerosis due to FUS mutation

(2009) Adriano Chiò et al.   NEUROBIOLOGY OF AGING

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

Broad clinical phenotypes associated with TAR-DNA binding protein (TARDBP) mutations in amyotrophic lateral sclerosis

(2009) Janine Kirby et al.   NEUROGENETICS

Mutations in FUS cause FALS and SALS in French and French Canadian populations

(2009) V. V. Belzil et al.   NEUROLOGY

ALS motor phenotype heterogeneity, focality, and spread: Deconstructing motor neuron degeneration

(2009) J. M. Ravits et al.   NEUROLOGY

Reduced expression of the Kinesin-Associated Protein 3 (KIFAP3) gene increases survival in sporadic amyotrophic lateral sclerosis

(2009) J. E. Landers et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Schwann cells expressing dismutase active mutant SOD1 unexpectedly slow disease progression in ALS mice

(2009) C. S. Lobsiger et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration

(2009) I. Wegorzewska et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Gamma and alpha motor neurons distinguished by expression of transcription factor Err3

(2009) A. Friese et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Subdivisions of primary motor cortex based on cortico-motoneuronal cells

(2009) Jean-Alban Rathelot et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6

(2009) C. Vance et al.   SCIENCE

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

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

Local-circuit phenotypes of layer 5 neurons in motor-frontal cortex of YFP-H mice

(2009) Jianing Yu   Frontiers in Neural Circuits

Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations

(2008) Ian R. A. Mackenzie et al.   ACTA NEUROPATHOLOGICA

Glycinergic Innervation of Motoneurons Is Deficient in Amyotrophic Lateral Sclerosis Mice

(2008) Qing Chang et al.   AMERICAN JOURNAL OF PATHOLOGY

Insulin-like growth factor-I for the treatment of amyotrophic lateral sclerosis

(2008) Stacey A. Sakowski et al.   Amyotrophic Lateral Sclerosis

TDP-43A315T mutation in familial motor neuron disease

(2008) Michael A. Gitcho et al.   ANNALS OF NEUROLOGY

Descending Pathways in Motor Control

(2008) Roger N. Lemon   Annual Review of Neuroscience

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

(2008) Felix Geser et al.   ARCHIVES OF NEUROLOGY

Cortical hyperexcitability may precede the onset of familial amyotrophic lateral sclerosis

(2008) Steve Vucic et al.   BRAIN

Identification of Positionally Distinct Astrocyte Subtypes whose Identities Are Specified by a Homeodomain Code

(2008) Christian Hochstim et al.   CELL

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

(2008) Hidefumi Ito et al.   EXPERIMENTAL NEUROLOGY

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 integrity of a lamin-B1-dependent nucleoskeleton is a fundamental determinant of RNA synthesis in human cells

(2008) C. W. Tang et al.   JOURNAL OF CELL SCIENCE

TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor

(2008) I.-Fan Wang et al.   JOURNAL OF NEUROCHEMISTRY

Schwann Cells Promote Synaptogenesis at the Neuromuscular Junction via Transforming Growth Factor- 1

(2008) Z. Feng et al.   JOURNAL OF NEUROSCIENCE

Neuron-Specific Expression of Mutant Superoxide Dismutase Is Sufficient to Induce Amyotrophic Lateral Sclerosis in Transgenic Mice

(2008) D. Jaarsma et al.   JOURNAL OF NEUROSCIENCE

The Semaphorin Receptor PlexinA3 Mediates Neuronal Apoptosis during Dorsal Root Ganglia Development

(2008) A. Ben-Zvi et al.   JOURNAL OF NEUROSCIENCE

TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysis

(2008) Vivianna M Van Deerlin et al.   LANCET NEUROLOGY

MnSOD deficiency has a differential effect on disease progression in two different ALS mutant mouse models

(2008) Florian L. Muller et al.   MUSCLE & NERVE

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

(2008) Edor Kabashi et al.   NATURE GENETICS

Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis

(2008) Koji Yamanaka et al.   NATURE NEUROSCIENCE

Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis

(2008) Julie D. Atkin et al.   NEUROBIOLOGY OF DISEASE

Upper motor neuron and extra-motor neuron involvement in amyotrophic lateral sclerosis: A clinical and brain imaging review

(2008) M.M. van der Graaff et al.   NEUROMUSCULAR DISORDERS

Mutant dynein (Loa) triggers proprioceptive axon loss that extends survival only in the SOD1 ALS model with highest motor neuron death

(2008) H. S. Ilieva et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2008) J. Sreedharan et al.   SCIENCE

Edaravone, a Free Radical Scavenger, Inhibits MMP-9–Related Brain Hemorrhage in Rats Treated With Tissue Plasminogen Activator

(2008) Kenji Yagi et al.   STROKE

Amyloid beta, mitochondrial dysfunction and synaptic damage: implications for cognitive decline in aging and Alzheimer's disease

(2008) P. Hemachandra Reddy et al.   TRENDS IN MOLECULAR MEDICINE

Novel Mutations in TARDBP (TDP-43) in Patients with Familial Amyotrophic Lateral Sclerosis

(2008) Nicola J. Rutherford et al.   PLoS Genetics

Parvalbumin overexpression alters immune-mediated increases in intracellular calcium, and delays disease onset in a transgenic model of familial amyotrophic lateral sclerosis

(2003) David R. Beers et al.   JOURNAL OF NEUROCHEMISTRY