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HUMAN MUTATION (2013)
TARDBP and FUS Mutations Associated with Amyotrophic Lateral Sclerosis: Summary and Update
Serena Lattante et al.
HUMAN MUTATION (2013)
Cognitive decline and reduced survival in C9orf72 expansion frontotemporal degeneration and amyotrophic lateral sclerosis
David J. Irwin et al.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2013)
Na+/K+ ATPase α1 and α3 Isoforms Are Differentially Expressed in α- and γ-Motoneurons
Ian J. Edwards et al.
JOURNAL OF NEUROSCIENCE (2013)
Expression of postsynaptic Ca2+-activated K+ (SK) channels at C-bouton synapses in mammalian lumbar α-motoneurons
Adam S. Deardorff et al.
JOURNAL OF PHYSIOLOGY-LONDON (2013)
Mitochondria, motor neurons and aging
M. L. Garcia et al.
JOURNAL OF THE NEUROLOGICAL SCIENCES (2013)
Association between repeat sizes and clinical and pathological characteristics in carriers of C90RF72 repeat expansions (Xpansize-72): a cross-sectional cohort study
Marka van Blitterswijk et al.
LANCET NEUROLOGY (2013)
Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS
Hong Joo Kim et al.
NATURE (2013)
Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis
Shin H. Kang et al.
NATURE NEUROSCIENCE (2013)
Amyotrophic lateral sclerosis-a model of corticofugal axonal spread
Heiko Braak et al.
NATURE REVIEWS NEUROLOGY (2013)
Explorative genetic study of UBQLN2 and PFN1 in an extended Flanders-Belgian cohort of frontotemporal lobar degeneration patients
Lubina Dillen et al.
NEUROBIOLOGY OF AGING (2013)
Lack of C9ORF72 coding mutations supports a gain of function for repeat expansions in amyotrophic lateral sclerosis
Matthew B. Harms et al.
NEUROBIOLOGY OF AGING (2013)
Neuroprotection through Excitability and mTOR Required in ALS Motoneurons to Delay Disease and Extend Survival
Smita Saxena et al.
NEURON (2013)
De Novo Mutations in Ataxin-2 Gene and ALS Risk
Jose Miguel Laffita-Mesa et al.
PLOS ONE (2013)
ALS-Associated TDP-43 Induces Endoplasmic Reticulum Stress, Which Drives Cytoplasmic TDP-43 Accumulation and Stress Granule Formation
Adam K. Walker et al.
PLOS ONE (2013)
Cortical energy demands of signaling and nonsignaling components in brain are conserved across mammalian species and activity levels
Fahmeed Hyder et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2013)
Cell and neuron densities in the primary motor cortex of primates
Nicole A. Young et al.
FRONTIERS IN NEURAL CIRCUITS (2013)
Heterogeneous neural coding of corrective movements in motor cortex
Adam S. Dickey et al.
FRONTIERS IN NEURAL CIRCUITS (2013)
In vivo optogenetic tracing of functional corticocortical connections between motor forelimb areas
Riichiro Hira et al.
FRONTIERS IN NEURAL CIRCUITS (2013)
Mapping the spatio-temporal structure of motor cortical LFP and spiking activities during reach-to-grasp movements
Alexa Riehle et al.
FRONTIERS IN NEURAL CIRCUITS (2013)
Prion-like Properties of Pathological TDP-43 Aggregates from Diseased Brains
Takashi Nonaka et al.
CELL REPORTS (2013)
A Neurodegeneration-Specific Gene-Expression Signature of Acutely Isolated Microglia from an Amyotrophic Lateral Sclerosis Mouse Model
Isaac M. Chiu et al.
CELL REPORTS (2013)
Eye tracking communication devices in amyotrophic lateral sclerosis: Impact on disability and quality of life
Marco Caligari et al.
AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION (2013)
SQSTM1 Mutations in French Patients With Frontotemporal Dementia or Frontotemporal Dementia With Amyotrophic Lateral Sclerosis
Isabelle Le Ber et al.
JAMA NEUROLOGY (2013)
Characterization of early pathogenesis in the SOD1G93A mouse model of ALS: part II, results and discussion
Sharon Vinsant et al.
BRAIN AND BEHAVIOR (2013)
Amyotrophic lateral sclerosis (ALS)-associated VAPB-P56S inclusions represent an ER quality control compartment
Marijn Kuijpers et al.
ACTA NEUROPATHOLOGICA COMMUNICATIONS (2013)
Clinical and pathological features of familial frontotemporal dementia caused by C9ORF72 mutation on chromosome 9p
Ging-Yuek R. Hsiung et al.
BRAIN (2012)
Frontotemporal dementia with the C9ORF72 hexanucleotide repeat expansion: clinical, neuroanatomical and neuropathological features
Colin J. Mahoney et al.
BRAIN (2012)
Transmissible Proteins: Expanding the Prion Heresy
Claudio Soto
CELL (2012)
The anatomy of cognitive impairment in amyotrophic lateral sclerosis: More than frontal lobe dysfunction
Stella Tsermentseli et al.
CORTEX (2012)
Regional spread pattern predicts survival in patients with sporadic amyotrophic lateral sclerosis
G. M. Gargiulo-Monachelli et al.
EUROPEAN JOURNAL OF NEUROLOGY (2012)
Transformation from a neuroprotective to a neurotoxic microglial phenotype in a mouse model of ALS
Bing Liao et al.
EXPERIMENTAL NEUROLOGY (2012)
Entorhinal cortical neurons are the primary targets of FUS mislocalization and ubiquitin aggregation in FUS transgenic rats
Cao Huang et al.
HUMAN MOLECULAR GENETICS (2012)
Evidence for an oligogenic basis of amyotrophic lateral sclerosis
Marka van Blitterswijk et al.
HUMAN MOLECULAR GENETICS (2012)
Reduced Calreticulin Levels Link Endoplasmic Reticulum Stress and Fas-Triggered Cell Death in Motoneurons Vulnerable to ALS
Nathalie Bernard-Marissal et al.
JOURNAL OF NEUROSCIENCE (2012)
Wnt7A Identifies Embryonic γ-Motor Neurons and Reveals Early Postnatal Dependence of γ-Motor Neurons on a Muscle Spindle-Derived Signal
Soha Ashrafi et al.
JOURNAL OF NEUROSCIENCE (2012)
Osteopontin Is an Alpha Motor Neuron Marker in the Mouse Spinal Cord
Hidemi Misawa et al.
JOURNAL OF NEUROSCIENCE RESEARCH (2012)
Glutamate modulates the firing rate in oculomotor nucleus motoneurons as a function of the recruitment threshold current
Julio Torres-Torrelo et al.
JOURNAL OF PHYSIOLOGY-LONDON (2012)
Sensorimotor function is modulated by the serotonin receptor 1d, a novel marker for gamma motor neurons
Anders Enjin et al.
MOLECULAR AND CELLULAR NEUROSCIENCE (2012)
Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study
Elisa Majounie et al.
LANCET NEUROLOGY (2012)
Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis
Chi-Hong Wu et al.
NATURE (2012)
EPHA4 is a disease modifier of amyotrophic lateral sclerosis in animal models and in humans
Annelies Van Hoecke et al.
NATURE MEDICINE (2012)
Mutant FUS induces endoplasmic reticulum stress in amyotrophic lateral sclerosis and interacts with protein disulfide-isomerase
Manal A. Farg et al.
NEUROBIOLOGY OF AGING (2012)
UNC13A is a modifier of survival in amyotrophic lateral sclerosis
Frank P. Diekstra et al.
NEUROBIOLOGY OF AGING (2012)
Length of normal alleles of C9ORF72 GGGGCC repeat do not influence disease phenotype
Nicola J. Rutherford et al.
NEUROBIOLOGY OF AGING (2012)
Screening in ALS and FTD patients reveals 3 novel UBQLN2 mutations outside the PXX domain and a pure FTD phenotype
Matthis Synofzik et al.
NEUROBIOLOGY OF AGING (2012)
UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis
Kelly L. Williams et al.
NEUROBIOLOGY OF AGING (2012)
Selective knockdown of mutant SOD1 in Schwann cells ameliorates disease in G85R mutant SOD1 transgenic mice
Lijun Wang et al.
NEUROBIOLOGY OF DISEASE (2012)
Profound downregulation of the RNA editing enzyme ADAR2 in ALS spinal motor neurons
Takuto Hideyama et al.
NEUROBIOLOGY OF DISEASE (2012)
SQSTM1 mutations in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Elisa Rubino et al.
NEUROLOGY (2012)
Shared Resistance to Aging and ALS in Neuromuscular Junctions of Specific Muscles
Gregorio Valdez et al.
PLOS ONE (2012)
A role for calpain-dependent cleavage of TDP-43 in amyotrophic lateral sclerosis pathology
Takenari Yamashita et al.
NATURE COMMUNICATIONS (2012)
Pathological heterogeneity in amyotrophic lateral sclerosis with FUS mutations: two distinct patterns correlating with disease severity and mutation
Ian R. A. Mackenzie et al.
ACTA NEUROPATHOLOGICA (2011)
Clinical and neuropathologic heterogeneity of c9FTD/ALS associated with hexanucleotide repeat expansion in C9ORF72
Melissa E. Murray et al.
ACTA NEUROPATHOLOGICA (2011)
Mutational Analysis Reveals the FUS Homolog TAF15 as a Candidate Gene for Familial Amyotrophic Lateral Sclerosis
N. Ticozzi et al.
AMERICAN JOURNAL OF MEDICAL GENETICS PART B-NEUROPSYCHIATRIC GENETICS (2011)
SQSTM1 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis
Faisal Fecto et al.
ARCHIVES OF NEUROLOGY (2011)
Blunting type 1 insulin-like growth factor receptor expression exacerbates neuronal apoptosis following hypoxic/ischemic injury
Wen Liu et al.
BMC NEUROSCIENCE (2011)
Endogenous regulatory T lymphocytes ameliorate amyotrophic lateral sclerosis in mice and correlate with disease progression in patients with amyotrophic lateral sclerosis
David R. Beers et al.
BRAIN (2011)
Morphological changes in nerve cells during normal aging
Ennio Pannese
BRAIN STRUCTURE & FUNCTION (2011)
Astrocyte loss of mutant SOD1 delays ALS disease onset and progression in G85R transgenic mice
Lijun Wang et al.
HUMAN MOLECULAR GENETICS (2011)
Ataxin-2 intermediate-length polyglutamine expansions in European ALS patients
Teresa Lee et al.
HUMAN MOLECULAR GENETICS (2011)
Ataxin-2 repeat-length variation and neurodegeneration
Owen A. Ross et al.
HUMAN MOLECULAR GENETICS (2011)
Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor κB-mediated pathogenic pathways
Vivek Swarup et al.
JOURNAL OF EXPERIMENTAL MEDICINE (2011)
The Frontotemporal Syndromes of ALS. Clinicopathological Correlates
Michael Joseph Strong et al.
JOURNAL OF MOLECULAR NEUROSCIENCE (2011)
Eye movements in amyotrophic lateral sclerosis and its mimics: a review with illustrative cases
Colette Donaghy et al.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2011)
Onset and spreading patterns of lower motor neuron involvements predict survival in sporadic amyotrophic lateral sclerosis
Chieko Fujimura-Kiyono et al.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2011)
Spinal Inhibitory Interneuron Pathology Follows Motor Neuron Degeneration Independent of Glial Mutant Superoxide Dismutase 1 Expression in SOD1-ALS Mice
Mehdi Hossaini et al.
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2011)
Pitx2 regulates myosin heavy chain isoform expression and multi-innervation in extraocular muscle
Yuefang Zhou et al.
JOURNAL OF PHYSIOLOGY-LONDON (2011)
Amyotrophic lateral sclerosis
Matthew C. Kiernan et al.
LANCET (2011)
Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia
Han-Xiang Deng et al.
NATURE (2011)
Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43
Magdalini Polymenidou et al.
NATURE NEUROSCIENCE (2011)
NOVEL p.Ile151Val MUTATION IN VCP IN A PATIENT OF AFRICAN AMERICAN DESCENT WITH SPORADIC ALS
M. DeJesus-Hernandez et al.
NEUROLOGY (2011)
Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS
Mariely DeJesus-Hernandez et al.
NEURON (2011)
A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD
Alan E. Renton et al.
NEURON (2011)
Misfolded SOD1 Associated with Motor Neuron Mitochondria Alters Mitochondrial Shape and Distribution Prior to Clinical Onset
Christine Vande Velde et al.
PLOS ONE (2011)
Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivo
Sophia T. Papadeas et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
Intermolecular transmission of superoxide dismutase 1 misfolding in living cells
Leslie I. Grad et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells
Christian Muench et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
The Unfolded Protein Response: From Stress Pathway to Homeostatic Regulation
Peter Walter et al.
SCIENCE (2011)
The endoplasmic reticulum and protein trafficking in dendrites and axons
Omar A. Ramirez et al.
TRENDS IN CELL BIOLOGY (2011)
TDP-43 pathology in sporadic ALS occurs in motor neurons lacking the RNA editing enzyme ADAR2
Hitoshi Aizawa et al.
ACTA NEUROPATHOLOGICA (2010)
FUS-Immunoreactive Inclusions Are a Common Feature in Sporadic and Non-SOD1 Familial Amyotrophic Lateral Sclerosis
Han-Xiang Deng et al.
ANNALS OF NEUROLOGY (2010)
Motor Neuron Diversity in Development and Disease
Kevin C. Kanning et al.
ANNUAL REVIEW OF NEUROSCIENCE, VOL 33 (2010)
Effects of ALS-related SOD1 mutants on dynein- and KIF5-mediated retrograde and anterograde axonal transpor
Ping Shi et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2010)
Protein disulphide isomerase protects against protein aggregation and is S-nitrosylated in amyotrophic lateral sclerosis
Adam K. Walker et al.
BRAIN (2010)
Global gene expression profiling of somatic motor neuron populations with different vulnerability identify molecules and pathways of degeneration and protection
Eva Hedlund et al.
BRAIN (2010)
Retrograde influence of muscle fibers on their innervation revealed by a novel marker for slow motoneurons
Joe V. Chakkalakal et al.
DEVELOPMENT (2010)
Neuropilin 1 Directly Interacts with Fer Kinase to Mediate Semaphorin 3A-induced Death of Cortical Neurons
Susan X. Jiang et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2010)
Identification of Novel Spinal Cholinergic Genetic Subtypes Disclose Chodl and Pitx2 as Markers for Fast Motor Neurons and Partition Cells
Anders Enjin et al.
JOURNAL OF COMPARATIVE NEUROLOGY (2010)
FUS mutations in amyotrophic lateral sclerosis: clinical, pathological, neurophysiological and genetic analysis
Ian P. Blair et al.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2010)
Upregulation of persistent sodium conductances in familial ALS
Steve Vucic et al.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2010)
Endoplasmic Reticulum Stress in Motor Neurons of the Spinal Cord in Sporadic Amyotrophic Lateral Sclerosis
Shoichi Sasaki
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2010)
Induced Loss of ADAR2 Engenders Slow Death of Motor Neurons from Q/R Site-Unedited GluR2
Takuto Hideyama et al.
JOURNAL OF NEUROSCIENCE (2010)
Wild-Type Human TDP-43 Expression Causes TDP-43 Phosphorylation, Mitochondrial Aggregation, Motor Deficits, and Early Mortality in Transgenic Mice
Ya-Fei Xu et al.
JOURNAL OF NEUROSCIENCE (2010)
Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS
Andrew C. Elden et al.
NATURE (2010)
Mutations of optineurin in amyotrophic lateral sclerosis
Hirofumi Maruyama et al.
NATURE (2010)
Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS
Daryl A. Bosco et al.
NATURE NEUROSCIENCE (2010)
Prion-like transmission of protein aggregates in neurodegenerative diseases
Patrik Brundin et al.
NATURE REVIEWS MOLECULAR CELL BIOLOGY (2010)
Broad clinical phenotypes associated with TAR-DNA binding protein (TARDBP) mutations in amyotrophic lateral sclerosis
Janine Kirby et al.
NEUROGENETICS (2010)
Misfolded Mutant SOD1 Directly Inhibits VDAC1 Conductance in a Mouse Model of Inherited ALS
Adrian Israelson et al.
NEURON (2010)
Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS
Jane O. Johnson et al.
NEURON (2010)
Questioning on the role of D amino acid oxidase in familial amyotrophic lateral sclerosis
Stephanie Millecamps et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2010)
Familial amyotrophic lateral sclerosis is associated with a mutation in D-amino acid oxidase
John Mitchell et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2010)
Deficits in axonal transport precede ALS symptoms in vivo
Lynsey G. Bilsland et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2010)
Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice
Xiu Shan et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2010)
TARDBP Mutations in Frontotemporal Lobar Degeneration: Frequency, Clinical Features, and Disease Course
B. Borroni et al.
REJUVENATION RESEARCH (2010)
Selective neuronal vulnerability to oxidative stress in the brain
Xinkun Wang et al.
FRONTIERS IN AGING NEUROSCIENCE (2010)
Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations
Ian R. A. Mackenzie et al.
ACTA NEUROPATHOLOGICA (2009)
Accumulation of TDP-43 and α-actin in an amyotrophic lateral sclerosis patient with the K17I ANG mutation
Danielle Seilhean et al.
ACTA NEUROPATHOLOGICA (2009)
Longitudinal study of muscle strength, quality, and adipose tissue infiltration
Matthew J. Delmonico et al.
AMERICAN JOURNAL OF CLINICAL NUTRITION (2009)
Glycinergic Innervation of Motoneurons Is Deficient in Amyotrophic Lateral Sclerosis Mice A Quantitative Confocal Analysis
Qing Chang et al.
AMERICAN JOURNAL OF PATHOLOGY (2009)
Insulin-like growth factor-I for the treatment of amyotrophic lateral sclerosis
Stacey A. Sakowski et al.
AMYOTROPHIC LATERAL SCLEROSIS (2009)
Axonal Mitochondrial Clusters Containing Mutant SOD1 in Transgenic Models of ALS
Jose R. Sotelo-Silveira et al.
ANTIOXIDANTS & REDOX SIGNALING (2009)
Genomic and biochemical approaches in the discovery of mechanisms for selective neuronal vulnerability to oxidative stress
Xinkun Wang et al.
BMC NEUROSCIENCE (2009)
A new subtype of frontotemporal lobar degeneration with FUS pathology
Manuela Neumann et al.
BRAIN (2009)
Increase in presynaptic territory of C-terminals on lumbar motoneurons of G93A SOD1 mice during disease progression
A. H. Pullen et al.
EUROPEAN JOURNAL OF NEUROSCIENCE (2009)
Depletion of TDP-43 affects Drosophila motoneurons terminal synapsis and locomotive behavior
Fabian Feiguin et al.
FEBS LETTERS (2009)
Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond
Hristelina Ilieva et al.
JOURNAL OF CELL BIOLOGY (2009)
Lamin B1 controls oxidative stress responses via Oct-1
Ashraf N. Malhas et al.
JOURNAL OF CELL BIOLOGY (2009)
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
Hiroaki Suzuki et al.
JOURNAL OF NEUROCHEMISTRY (2009)
Early Excitability Changes in Lumbar Motoneurons of Transgenic SOD1G85R and SOD1G93A-Low Mice
Arnaud Pambo-Pambo et al.
JOURNAL OF NEUROPHYSIOLOGY (2009)
Progressive Changes in Synaptic Inputs to Motoneurons in Adult Sacral Spinal Cord of a Mouse Model of Amyotrophic Lateral Sclerosis
Mingchen Jiang et al.
JOURNAL OF NEUROSCIENCE (2009)
A Switch in Retrograde Signaling from Survival to Stress in Rapid-Onset Neurodegeneration
Eran Perlson et al.
JOURNAL OF NEUROSCIENCE (2009)
A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice
Smita Saxena et al.
NATURE NEUROSCIENCE (2009)
Gamma motor neurons express distinct genetic markers at birth and require muscle spindle-derived GDNF for postnatal survival
Neil A. Shneider et al.
NEURAL DEVELOPMENT (2009)
Two Italian kindreds with familial amyotrophic lateral sclerosis due to FUS mutation
Adriano Chio et al.
NEUROBIOLOGY OF AGING (2009)
The effect of mutant SOD1 dismutase activity on non-cell autonomous degeneration in familial amyotrophic lateral sclerosis
Lijun Wang et al.
NEUROBIOLOGY OF DISEASE (2009)
Mutations in FUS cause FALS and SALS in French and French Canadian populations
V. V. Belzil et al.
NEUROLOGY (2009)
ALS motor phenotype heterogeneity, focality, and spread Deconstructing motor neuron degeneration
John M. Ravits et al.
NEUROLOGY (2009)
Upper motor neuron and extra-motor neuron involvement in amyotrophic lateral sclerosis: A clinical and brain imaging review
M. M. van der Graaff et al.
NEUROMUSCULAR DISORDERS (2009)
Reduced expression of the Kinesin-Associated Protein 3 (KIFAP3) gene increases survival in sporadic amyotrophic lateral sclerosis
John E. Landers et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
Schwann cells expressing dismutase active mutant SOD1 unexpectedly slow disease progression in ALS mice
Christian S. Lobsiger et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration
Iga Wegorzewska et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
Gamma and alpha motor neurons distinguished by expression of transcription factor Err3
Andreas Friese et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
Subdivisions of primary motor cortex based on cortico-motoneuronal cells
Jean-Alban Rathelot et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6
Caroline Vance et al.
SCIENCE (2009)
Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis
T. J. Kwiatkowski et al.
SCIENCE (2009)
Edaravone, a Free Radical Scavenger, Inhibits MMP-9-Related Brain Hemorrhage in Rats Treated With Tissue Plasminogen Activator
Kenji Yagi et al.
STROKE (2009)
Local-circuit phenotypes of layer 5 neurons in motor-frontal cortex of YFP-H mice
Jianing Yu
Frontiers in Neural Circuits (2009)
TDP-43 A315T mutation in familial motor neuron disease
Michael A. Gitcho et al.
ANNALS OF NEUROLOGY (2008)
Descending pathways in motor control
Roger N. Lemon
ANNUAL REVIEW OF NEUROSCIENCE (2008)
Evidence of multisystem disorder in whole-brain map of pathological TDP-43 in amyotrophic lateral sclerosis
Felix Geser et al.
ARCHIVES OF NEUROLOGY (2008)
Cortical hyperexcitability may precede the onset of familial amyotrophic lateral sclerosis
Steve Vucic et al.
BRAIN (2008)
Identification of positionally distinct astrocyte subtypes whose identities are specified by a homeodomain code
Christian Hochstim et al.
CELL (2008)
Treatment with edaravone, initiated at symptom onset, slows motor decline and decreases SOD1 deposition in ALS mice
Hidefumi Ito et al.
EXPERIMENTAL NEUROLOGY (2008)
ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1
Hideki Nishitoh et al.
GENES & DEVELOPMENT (2008)
The integrity of a lamin-B1-dependent nucleoskeleton is a fundamental determinant of RNA synthesis in human cells
Chi W. Tang et al.
JOURNAL OF CELL SCIENCE (2008)
TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor
I. -Fan Wang et al.
JOURNAL OF NEUROCHEMISTRY (2008)
Schwann cells promote synaptogenesis at the neuromuscular junction via transforming growth factor-β1
Zhihua Feng et al.
JOURNAL OF NEUROSCIENCE (2008)
Neuron-specific expression of mutant superoxide dismutase is sufficient to induce amyotrophic lateral sclerosis in transgenic mice
Dick Jaarsma et al.
JOURNAL OF NEUROSCIENCE (2008)
The Semaphorin Receptor PlexinA3 Mediates Neuronal Apoptosis during Dorsal Root Ganglia Development
Ayal Ben-Zvi et al.
JOURNAL OF NEUROSCIENCE (2008)
TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology:: a genetic and histopathological analysis
Vivianna M. Van Deerlin et al.
LANCET NEUROLOGY (2008)
MnSOD deficiency has a differential effect on disease progression in two different ALS mutant mouse models
Florian L. Muller et al.
MUSCLE & NERVE (2008)
TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis
Edor Kabashi et al.
NATURE GENETICS (2008)
Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis
Koji Yamanaka et al.
NATURE NEUROSCIENCE (2008)
Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis
Julie D. Atkin et al.
NEUROBIOLOGY OF DISEASE (2008)
Mutant dynein (Loa) triggers proprioceptive axon loss that extends survival only in the SOD1 ALS model with highest motor neuron death
Hristelina S. Ilieva et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2008)
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis
Jemeen Sreedharan et al.
SCIENCE (2008)
Amyloid beta, mitochondrial dysfunction and synaptic damage: implications for cognitive decline in aging and Alzheimer's disease
P. Hemachandra Reddy et al.
TRENDS IN MOLECULAR MEDICINE (2008)
Novel Mutations in TARDBP(TDP-43) in Patients with Familial Amyotrophic Lateral Sclerosis
Nicola J. Rutherford et al.
PLOS GENETICS (2008)
Adult glial precursor proliferation in mutant SOD1G93A mice
Tim Magnus et al.
GLIA (2008)
Altered quality control in the endoplasmic reticulum causes cortical dysplasia in knock-in mice expressing a mutant BiP
Naoya Mimura et al.
MOLECULAR AND CELLULAR BIOLOGY (2008)
TDP-43-immunoreactive neuronal and glial inclusions in the neostriatum in amyotrophic lateral sclerosis with and without dementia
Haixin Zhang et al.
ACTA NEUROPATHOLOGICA (2008)
Proprioceptive sensory neuropathy in mice with a mutation in the cytoplasmic dynein heavy chain 1 gene
Xiang-Jun Chen et al.
JOURNAL OF NEUROSCIENCE (2007)
Semaphorin and neuropilin co-expression in motoneurons sets axon sensitivity to environmental semaphorin sources during motor axon pathfinding
Frederic Moret et al.
DEVELOPMENT (2007)
Angiogenin loss-of-function mutations in amyotrophic lateral sclerosis
David Wu et al.
ANNALS OF NEUROLOGY (2007)
Mitochondrial alterations in dorsal root ganglion cells in sporadic amyotrophic lateral sclerosis
Shoichi Sasaki et al.
ACTA NEUROPATHOLOGICA (2007)
Oxidative and endoplasmic reticulum stress interplay in sporadic amyotrophic lateral sclerosis
Ekaterina V. Ilieva et al.
BRAIN (2007)
Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content
Kurt J. De Vos et al.
HUMAN MOLECULAR GENETICS (2007)
Time course of preferential motor unit loss in the SODIG93A mouse model of amyotrophic lateral sclerosis
J. Hegedus et al.
NEUROBIOLOGY OF DISEASE (2007)
Differential expression of molecular motors in the motor cortex of sporadic ALS
Maria Pantelidou et al.
NEUROBIOLOGY OF DISEASE (2007)
Focality of upper and lower motor neuron degeneration at the clinical onset of ALS
John Ravits et al.
NEUROLOGY (2007)
TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation
Chun-Feng Tan et al.
ACTA NEUROPATHOLOGICA (2007)
Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons
Makiko Nagai et al.
NATURE NEUROSCIENCE (2007)
Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations
Ian R. A. Mackenzie et al.
ANNALS OF NEUROLOGY (2007)
Therapeutic immunization with a glatiramer acetate derivative does not alter survival in G93A and G37R SOD1 mouse models of familial ALS
Christine Haenggeli et al.
NEUROBIOLOGY OF DISEASE (2007)
Differentiation between primary lateral sclerosis and amyotrophic lateral sclerosis - Examination of symptoms and signs at disease onset and during follow-up
Maria Carmela Tartaglia et al.
ARCHIVES OF NEUROLOGY (2007)
Early electrophysiological abnormalities in lumbar motoneurons in a transgenic mouse model of amyotrophic lateral sclerosis
Cyril Bories et al.
EUROPEAN JOURNAL OF NEUROSCIENCE (2007)
Interaction between familial amyotrophic lateral sclerosis (ALS)-linked SOD1 mutants and the dynein complex
Fujian Zhang et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2007)
TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Tetsuaki Arai et al.
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2006)
Investigation of the therapeutic effects of edaravone, a free radical scavenger, on amyotrophic lateral sclerosis (Phase II study)
Hiide Yoshino et al.
AMYOTROPHIC LATERAL SCLEROSIS (2006)
Motor neuron disease in mice expressing the wild type-like D90A mutant superoxide dismutase-1
P. Andreas Jonsson et al.
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2006)
The role of excitotoxicity in the pathogenesis of amyotrophic lateral sclerosis
L. Van den Bosch et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2006)
Axotomy induces contrasting changes in calcium and calcium-binding proteins in oculomotor and hypoglossal nuclei of balb/c mice
Izabella Obal et al.
JOURNAL OF COMPARATIVE NEUROLOGY (2006)
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Manuela Neumann et al.
SCIENCE (2006)
ALS:: A disease of motor neurons and their nonneuronal neighbors
Sverine Boillee et al.
NEURON (2006)
In vivo quantification of spinal and bulbar motor neuron degeneration in the G93A-SOD1 transgenic mouse model of ALS by T2 relaxation time and apparent diffusion coefficient
Heiko G. Niessen et al.
EXPERIMENTAL NEUROLOGY (2006)
Familial ALS-superoxide dismutases associate with mitochondria and shift their redox potentials
Alberto Ferri et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2006)
Defective axonal transport of neurofilament proteins in neurons overexpressing peripherin
Stephanie Millecamps et al.
JOURNAL OF NEUROCHEMISTRY (2006)
Onset and progression in inherited ALS determined by motor neurons and microglia
Severine Boillee et al.
SCIENCE (2006)
Widespread loss of neuronal populations in the spinal ventral horn in sporadic motor neuron disease. A morphometric study
Benjamin Stephens et al.
JOURNAL OF THE NEUROLOGICAL SCIENCES (2006)
The expression of the chemorepellent Semaphorin 3A is selectively induced in terminal Schwann cells of a subset of neuromuscular synapses that display limited anatomical plasticity and enhanced vulnerability in motor neuron disease
Fred De Winter et al.
MOLECULAR AND CELLULAR NEUROSCIENCE (2006)
Synaptic mitochondria are more susceptible to Ca2+ overload than nonsynaptic mitochondria
MR Brown et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2006)
Parvalburnin and calbindin D-28k immunoreactivity in transgenic mice with a G93A mutant SOD1 gene
S Sasaki et al.
BRAIN RESEARCH (2006)
Ageing and neuronal vulnerability
MP Mattson et al.
NATURE REVIEWS NEUROSCIENCE (2006)
ANG mutations segregate with familial and 'sporadic' amyotrophic lateral sclerosis
MJ Greenway et al.
NATURE GENETICS (2006)
Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF
S Pun et al.
NATURE NEUROSCIENCE (2006)
Distinct roles for secreted semaphorin signaling in spinal motor axon guidance
AB Huber et al.
NEURON (2005)
Heterozygous R1101K mutation of the DCTN1 gene in a family with ALS and FTD
C Münch et al.
ANNALS OF NEUROLOGY (2005)
Astrocytes, from brain glue to communication elements: The revolution continues
A Volterra et al.
NATURE REVIEWS NEUROSCIENCE (2005)
Expression profile of AMPA receptor subunit mRNA in single adult rat brain and spinal cord neurons in situ
H Sun et al.
NEUROSCIENCE RESEARCH (2005)
Age-related changes in regional brain mitochondria from Fischer 344 rats
R LaFrance et al.
AGING CELL (2005)
Mutant superoxide dismutase disrupts cytoplasmic dynein in motor neurons
LA Ligon et al.
NEUROREPORT (2005)
Mammalian motor neurons corelease glutamate and acetylcholine at central synapses
H Nishimaru et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2005)
A selective inhibitor-of eIF2α dephosphorylation protects cells from ER stress
M Boyce et al.
SCIENCE (2005)
Gene expression profile of spinal motor neurons in sporadic amyotrophic lateral sclerosis
YM Jiang et al.
ANNALS OF NEUROLOGY (2005)
Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man
LR Fischer et al.
EXPERIMENTAL NEUROLOGY (2004)
Expression of peripherin in ubiquinated inclusions of amyotrophic lateral sclerosis
CZ He et al.
JOURNAL OF THE NEUROLOGICAL SCIENCES (2004)
Cytoplasmic dynein regulates the subcellular distribution of mitochondria by controlling the recruitment of the fission factor dynamin-related protein-1
A Varadi et al.
JOURNAL OF CELL SCIENCE (2004)
Point mutations of the p150 subunit of dynactin (DCTN1) gene in ALS
C Münch et al.
NEUROLOGY (2004)
NIMA-related protein kinase 1 is involved early in the ionizing radiation-induced DNA damage response
R Polci et al.
CANCER RESEARCH (2004)
Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria
J Liu et al.
NEURON (2004)
Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria
P Pasinelli et al.
NEURON (2004)
Thinning of the cerebral cortex in aging
DH Salat et al.
CEREBRAL CORTEX (2004)
Organization of rat vibrissa motor cortex and adjacent areas according to cytoarchitectonics, microstimulation, and intracellular stimulation of identified cells
M Brecht et al.
JOURNAL OF COMPARATIVE NEUROLOGY (2004)
Effect of transgene copy number on survival in the G93A SOD1 transgenic mouse model of ALS
GM Alexander et al.
MOLECULAR BRAIN RESEARCH (2004)
A mutation in the vesicle-trafficking protein VAPB causes late-onset spinal muscular atrophy and amyotrophic lateral sclerosis
AL Nishimura et al.
AMERICAN JOURNAL OF HUMAN GENETICS (2004)
Cortical selective vulnerability in motor neuron disease: a morphometric study
S Maekawa et al.
BRAIN (2004)
Calcium-permeable AMPA receptors promote misfolding of mutant SOD1 protein and development of amyotrophic lateral sclerosis in a transgenic mouse model
M Tateno et al.
HUMAN MOLECULAR GENETICS (2004)
Glial cells maintain synaptic structure and function and promote development of the neuromuscular junction in vivo
LV Reddy et al.
NEURON (2003)
Neurodegeneration in amyotrophic lateral sclerosis:: the role of oxidative stress and altered homeostasis of metals
MT Carrì et al.
BRAIN RESEARCH BULLETIN (2003)
Marked loss of myelinated nerve fibers in the human brain with age
L Marner et al.
JOURNAL OF COMPARATIVE NEUROLOGY (2003)
Sixteen novel mutations in the Cu/Zn superoxide dismutase gene in amyotrophic lateral sclerosis: a decade of discoveries, defects and disputes
PM Andersen et al.
AMYOTROPHIC LATERAL SCLEROSIS AND OTHER MOTOR NEURON DISORDERS (2003)
Human spinal motoneurons express low relative abundance of GluR2 mRNA: an implication for excitotoxicity in ALS
Y Kawahara et al.
JOURNAL OF NEUROCHEMISTRY (2003)
Mutant dynactin in motor neuron disease
I Puls et al.
NATURE GENETICS (2003)
A neurotoxic peripherin splice variant in a mouse model of ALS
J Robertson et al.
JOURNAL OF CELL BIOLOGY (2003)
Mitochondrial dysfunction and death in motor neurons exposed to the glutathione-depleting agent ethacrynic acid
M Rizzardini et al.
JOURNAL OF THE NEUROLOGICAL SCIENCES (2003)
Mechanisms that contribute to differences in motor performance between young and old adults
RM Enoka et al.
JOURNAL OF ELECTROMYOGRAPHY AND KINESIOLOGY (2003)
Age-associated changes in skeletal muscles and their effect on mobility: an operational diagnosis of sarcopenia
F Lauretani et al.
JOURNAL OF APPLIED PHYSIOLOGY (2003)
Anti-semaphorin 3A antibodies rescue retinal ganglion cells from cell death following optic nerve axotomy
A Shirvan et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2002)
Control of cell survival by IGF signaling pathways
AM Vincent et al.
GROWTH HORMONE & IGF RESEARCH (2002)
Accumulation of SOD1 mutants in postnatal motoneurons does not cause motoneuron pathology or motoneuron disease
MM Lino et al.
JOURNAL OF NEUROSCIENCE (2002)
Disruption of dynein/dynactin inhibits axonal transport in motor neurons causing late-onset progressive degeneration
BH LaMonte et al.
NEURON (2002)
Mitochondrial DNA and respiratory chain function in spinal cords of ALS patients
FR Wiedemann et al.
JOURNAL OF NEUROCHEMISTRY (2002)
Association of chorea and motor neuron disease
PF Pradat et al.
MOVEMENT DISORDERS (2002)
Parvalbumin overexpression alters immune-mediated increases in intracellular calcium, and delays disease onset in a transgenic model of familial amyotrophic lateral sclerosis
DR Beers et al.
JOURNAL OF NEUROCHEMISTRY (2001)
Morphological evidence for lipid peroxidation and protein glycoxidation in spinal cords from sporadic amyotrophic lateral sclerosis patients
N Shibata et al.
BRAIN RESEARCH (2001)
Immune reactivity in a mouse model of familial ALS correlates with disease progression
ME Alexianu et al.
NEUROLOGY (2001)
Ubiquitin immunoreactivity in presumed spinal interneurones in motor neurone disease
B Stephens et al.
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY (2001)
Neuronal Cav1.3α1 L-type channels activate at relatively hyperpolarized membrane potentials and are incompletely inhibited by dihydropyridines
WF Xu et al.
JOURNAL OF NEUROSCIENCE (2001)
Specificity in ligand binding and intracellular signalling by insulin and insulin-like growth factor receptors
K Siddle et al.
BIOCHEMICAL SOCIETY TRANSACTIONS (2001)
RNA editing at the Q/R site for the glutamate receptor subunits GLUR2, GLUR5, and GLUR6 in hippocampus and temporal cortex from epileptic patients
G Kortenbruck et al.
NEUROBIOLOGY OF DISEASE (2001)
Neuron-specific expression of mutant superoxide dismutase 1 in transgenic mice does not lead to motor impairment
A Pramatarova et al.
JOURNAL OF NEUROSCIENCE (2001)
Different receptors mediate motor neuron death induced by short and long exposures to excitotoxicity
L Van den Bosch et al.
BRAIN RESEARCH BULLETIN (2000)
Calcium binding proteins in motoneurons at low and high risk for degeneration in ALS
P Laslo et al.
NEUROREPORT (2000)
Increased oxidative damage to DNA in ALS patients
M Bogdanov et al.
FREE RADICAL BIOLOGY AND MEDICINE (2000)
Mitochondrial DNA deletion mutation levels are elevated in ALS brains
GK Dhaliwal et al.
NEUROREPORT (2000)
Calcium dynamics and buffering in oculomotor neurones from mouse that are particularly resistant during amyotrophic lateral sclerosis (ALS)-related motoneurone disease
BK Vanselow et al.
JOURNAL OF PHYSIOLOGY-LONDON (2000)
KIF5C, a novel neuronal kinesin enriched in motor neurons
Y Kanai et al.
JOURNAL OF NEUROSCIENCE (2000)
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