Advances in Cellular Models to Explore the Pathophysiology of Amyotrophic Lateral Sclerosis

Oligodendrocyte dysfunction in the pathogenesis of amyotrophic lateral sclerosis

(2013) Thomas Philips et al.   BRAIN

Accelerated High-Yield Generation of Limb-Innervating Motor Neurons from Human Stem Cells

(2013) M. W. Amoroso et al.   JOURNAL OF NEUROSCIENCE

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

(2013) Shin H Kang et al.   NATURE NEUROSCIENCE

The C9orf72 GGGGCC Repeat Is Translated into Aggregating Dipeptide-Repeat Proteins in FTLD/ALS

(2013) K. Mori et al.   SCIENCE

Human Stem Cell-Derived Spinal Cord Astrocytes with Defined Mature or Reactive Phenotypes

(2013) Laurent Roybon et al.   Cell Reports

TDP-43 regulates the mammalian spinogenesis through translational repression of Rac1

(2012) Pritha Majumder et al.   ACTA NEUROPATHOLOGICA

A novel monoclonal antibody reveals a conformational alteration shared by amyotrophic lateral sclerosis-linked SOD1 mutants

(2012) Takao Fujisawa et al.   ANNALS OF NEUROLOGY

Substituted pyrazolones require N2 hydrogen bond donating ability to protect against cytotoxicity from protein aggregation of mutant superoxide dismutase 1

(2012) Paul C. Trippier et al.   BIOORGANIC & MEDICINAL CHEMISTRY LETTERS

Role of selected mutations in the Q/N rich region of TDP-43 in EGFP-12xQ/N-induced aggregate formation

(2012) Mauricio Budini et al.   BRAIN RESEARCH

Therapeutic Potential of N-Acetyl-Glucagon-Like Peptide-1 in Primary Motor Neuron Cultures Derived From Non-Transgenic and SOD1-G93A ALS Mice

(2012) Hui Sun et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

The unfolded protein response in models of human mutant G93A amyotrophic lateral sclerosis

(2012) T. Prell et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

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

(2012) Bing Liao et al.   EXPERIMENTAL NEUROLOGY

Extracellular aggregated Cu/Zn superoxide dismutase activates microglia to give a cytotoxic phenotype

(2012) Kate Roberts et al.   GLIA

Evidence for an oligogenic basis of amyotrophic lateral sclerosis

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

ALS-Associated Ataxin 2 PolyQ Expansions Enhance Stress-Induced Caspase 3 Activation and Increase TDP-43 Pathological Modifications

(2012) M. P. Hart et al.   JOURNAL OF NEUROSCIENCE

Motoneurons Secrete Angiogenin to Induce RNA Cleavage in Astroglia

(2012) A. Skorupa et al.   JOURNAL OF NEUROSCIENCE

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

A yeast model of optineurin proteinopathy reveals a unique aggregation pattern associated with cellular toxicity

(2012) Dmitry Kryndushkin et al.   MOLECULAR MICROBIOLOGY

Median nerve ultrasound as a screening tool in carpal tunnel syndrome: Correlation of cross-sectional area measures with electrodiagnostic abnormality

(2012) Justin T. Mhoon et al.   MUSCLE & NERVE

Oligodendroglia metabolically support axons and contribute to neurodegeneration

(2012) Youngjin Lee et al.   NATURE

Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs

(2012) Clotilde Lagier-Tourenne et al.   NATURE NEUROSCIENCE

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

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

(2012) Lijun Wang et al.   NEUROBIOLOGY OF DISEASE

Mutant SOD1G93A triggers mitochondrial fragmentation in spinal cord motor neurons: Neuroprotection by SIRT3 and PGC-1α

(2012) Wenjun Song et al.   NEUROBIOLOGY OF DISEASE

Opposite regulation of metabotropic glutamate receptor 3 and metabotropic glutamate receptor 5 by inflammatory stimuli in cultured microglia and astrocytes

(2012) J.V. Berger et al.   NEUROSCIENCE

Dysregulation of iron protein expression in the G93A model of amyotrophic lateral sclerosis

(2012) M. Hadzhieva et al.   NEUROSCIENCE

Ablation of Proliferating Cells in the CNS Exacerbates Motor Neuron Disease Caused by Mutant Superoxide Dismutase

(2012) Jean-Nicolas Audet et al.   PLoS One

Aberrant Localization of FUS and TDP43 Is Associated with Misfolding of SOD1 in Amyotrophic Lateral Sclerosis

(2012) Edward Pokrishevsky et al.   PLoS One

Bim Links ER Stress and Apoptosis in Cells Expressing Mutant SOD1 Associated with Amyotrophic Lateral Sclerosis

(2012) Kai Y. Soo et al.   PLoS One

Modulation of Astrocytic Mitochondrial Function by Dichloroacetate Improves Survival and Motor Performance in Inherited Amyotrophic Lateral Sclerosis

(2012) Ernesto Miquel et al.   PLoS One

Creation of an Open-Access, Mutation-Defined Fibroblast Resource for Neurological Disease Research

(2012) Selina Wray et al.   PLoS One

Monitoring CSF Proteome Alterations in Amyotrophic Lateral Sclerosis: Obstacles and Perspectives in Translating a Novel Marker Panel to the Clinic

(2012) Nils von Neuhoff et al.   PLoS One

An over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1

(2012) S. Guareschi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Drug Screening for ALS Using Patient-Specific Induced Pluripotent Stem Cells

(2012) N. Egawa et al.   Science Translational Medicine

L-BMAA Induced ER Stress and Enhanced Caspase 12 Cleavage in Human Neuroblastoma SH-SY5Y Cells at Low Nonexcitotoxic Concentrations

(2012) Oliver Okle et al.   TOXICOLOGICAL SCIENCES

Are environmental exposures to selenium, heavy metals, and pesticides risk factors for amyotrophic lateral sclerosis?

(2012) Marco Vinceti et al.   REVIEWS ON ENVIRONMENTAL HEALTH

A mutation in sigma-1 receptor causes juvenile amyotrophic lateral sclerosis

(2011) Amr Al-Saif et al.   ANNALS OF NEUROLOGY

Smoking and Risk of Amyotrophic Lateral Sclerosis

(2011) Hao Wang et al.   ARCHIVES OF NEUROLOGY

Sterol regulatory element binding protein-1 (SREBP1) activation in motor neurons in excitotoxicity and amyotrophic lateral sclerosis (ALS): Indip, a potential therapeutic peptide

(2011) Changiz Taghibiglou et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Neuromuscular junction formation between human stem cell-derived motoneurons and human skeletal muscle in a defined system

(2011) Xiufang Guo et al.   BIOMATERIALS

Phosphatase and tensin homologue/protein kinase B pathway linked to motor neuron survival in human superoxide dismutase 1-related amyotrophic lateral sclerosis

(2011) Janine Kirby et al.   BRAIN

Brain Energy Metabolism: Focus on Astrocyte-Neuron Metabolic Cooperation

(2011) Mireille Bélanger et al.   Cell Metabolism

Motor neuron impairment mediated by a sumoylated fragment of the glial glutamate transporter EAAT2

(2011) Emily Foran et al.   GLIA

Downregulation of VAPB expression in motor neurons derived from induced pluripotent stem cells of ALS8 patients

(2011) Miguel Mitne-Neto et al.   HUMAN MOLECULAR GENETICS

Alsin and SOD1G93AProteins Regulate Endosomal Reactive Oxygen Species Production by Glial Cells and Proinflammatory Pathways Responsible for Neurotoxicity

(2011) Qiang Li et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Muscle cells and motoneurons differentially remove mutant SOD1 causing familial amyotrophic lateral sclerosis

(2011) Elisa Onesto et al.   JOURNAL OF NEUROCHEMISTRY

Autophagy in Spinal Cord Motor Neurons in Sporadic Amyotrophic Lateral Sclerosis

(2011) Shoichi Sasaki   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Differential effect of oxidative or excitotoxic stress on the transcriptional profile of amyotrophic lateral sclerosis-linked mutant SOD1 cultured neurons

(2011) Nadia Boutahar et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Neuroinflammation in amyotrophic lateral sclerosis: role of glial activation in motor neuron disease

(2011) Thomas Philips et al.   LANCET NEUROLOGY

C-Jun N-terminal kinase controls TDP-43 accumulation in stress granules induced by oxidative stress

(2011) Jodi Meyerowitz et al.   Molecular Neurodegeneration

Astrocytes from familial and sporadic ALS patients are toxic to motor neurons

(2011) Amanda M Haidet-Phillips et al.   NATURE BIOTECHNOLOGY

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

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

Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis

(2011) Laura Ferraiuolo et al.   Nature Reviews Neurology

Substance P receptor activation induces downregulation of the AMPA receptor functionality in cortical neurons from a genetic model of Amyotrophic Lateral Sclerosis

(2011) Silvia Caioli et al.   NEUROBIOLOGY OF DISEASE

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

Exposure of neurons to excitotoxic levels of glutamate induces cleavage of the RNA editing enzyme, adenosine deaminase acting on RNA 2, and loss of GLUR2 editing

(2011) S.S. Mahajan et al.   NEUROSCIENCE

Protective effects of resveratrol through the up-regulation of SIRT1 expression in the mutant hSOD1-G93A-bearing motor neuron-like cell culture model of amyotrophic lateral sclerosis

(2011) Jing Wang et al.   NEUROSCIENCE LETTERS

Disease-Related Changes in the Cerebrospinal Fluid Metabolome in Amyotrophic Lateral Sclerosis Detected by GC/TOFMS

(2011) Anna Wuolikainen et al.   PLoS One

Physiological characterization of human muscle acetylcholine receptors from ALS patients

(2011) E. Palma et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Linking hypoxic and oxidative insults to cell death mechanisms in models of ALS

(2010) Renshi Xu et al.   BRAIN RESEARCH

Modulation of Synaptic Transmission and Analysis of Neuroprotective Effects of Valproic Acid and Derivates in Rat Embryonic Motoneurons

(2010) D. Ragancokova et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Metabolomic analysis of serum by (1) H NMR spectroscopy in amyotrophic lateral sclerosis

(2010) Alok Kumar et al.   CLINICA CHIMICA ACTA

Zinc induces depletion and aggregation of endogenous TDP-43

(2010) Aphrodite Caragounis et al.   FREE RADICAL BIOLOGY AND MEDICINE

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

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

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

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

Extracellular ATP and the P2X7 receptor in astrocyte-mediated motor neuron death: implications for amyotrophic lateral sclerosis

(2010) Mandi Gandelman et al.   Journal of Neuroinflammation

An estimate of amyotrophic lateral sclerosis heritability using twin data

(2010) A. Al-Chalabi et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Collapsin Response Mediator Protein 4a (CRMP4a) Is Upregulated in Motoneurons of Mutant SOD1 Mice and Can Trigger Motoneuron Axonal Degeneration and Cell Death

(2010) L. Duplan et al.   JOURNAL OF NEUROSCIENCE

1H-NMR-Based Metabolomic Profiling of CSF in Early Amyotrophic Lateral Sclerosis

(2010) Hélène Blasco et al.   PLoS One

Impairment of mitochondrial calcium handling in a mtSOD1 cell culture model of motoneuron disease

(2009) Manoj Jaiswal et al.   BMC NEUROSCIENCE

Angiogenin protects motoneurons against hypoxic injury

(2009) J Sebastià et al.   CELL DEATH AND DIFFERENTIATION

TDP-43 Dimerizes in Human Cells in Culture

(2009) Yuki Shiina et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

An in vitro screening cascade to identify neuroprotective antioxidants in ALS

(2009) Siân C. Barber et al.   FREE RADICAL BIOLOGY AND MEDICINE

Oxidative stress in ALS: Key role in motor neuron injury and therapeutic target

(2009) Siân C. Barber et al.   FREE RADICAL BIOLOGY AND MEDICINE

Extracellular mutant SOD1 induces microglial-mediated motoneuron injury

(2009) Weihua Zhao et al.   GLIA

Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo

(2009) Edor Kabashi et al.   HUMAN MOLECULAR GENETICS

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

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

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

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

Isolation and enrichment of embryonic mouse motoneurons from the lumbar spinal cord of individual mouse embryos

(2009) Stefan Wiese et al.   Nature Protocols

VEGF protects motor neurons against excitotoxicity by upregulation of GluR2

(2009) Elke Bogaert et al.   NEUROBIOLOGY OF AGING

Lead exposure stimulates VEGF expression in the spinal cord and extends survival in a mouse model of ALS

(2009) Ana G. Barbeito et al.   NEUROBIOLOGY OF DISEASE

Mutant SOD1 G93A microglia have an inflammatory phenotype and elevated production of MCP-1

(2009) Siranush A. Sargsyan et al.   NEUROREPORT

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

Mécanismes de dérégulation de la réponse à l’hypoxie dans la sclérose latérale amyotrophique

(2009) C. Moreau et al.   REVUE NEUROLOGIQUE

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

Non-Cell-Autonomous Effect of Human SOD1G37R Astrocytes on Motor Neurons Derived from Human Embryonic Stem Cells

(2008) Maria C.N. Marchetto et al.   Cell Stem Cell

Human Embryonic Stem Cell-Derived Motor Neurons Are Sensitive to the Toxic Effect of Glial Cells Carrying an ALS-Causing Mutation

(2008) Francesco Paolo Di Giorgio et al.   Cell Stem Cell

Oxidative and excitotoxic insults exert differential effects on spinal motoneurons and astrocytic glutamate transporters: Implications for the role of astrogliosis in amyotrophic lateral sclerosis

(2008) Chrissandra J. Zagami et al.   GLIA

Expression of mutant SOD1G93Ain astrocytes induces functional deficits in motoneuron mitochondria

(2008) Lynsey G. Bilsland et al.   JOURNAL OF NEUROCHEMISTRY

Recruitment of mitochondria into apoptotic signaling correlates with the presence of inclusions formed by amyotrophic lateral sclerosis-associated SOD1 mutations

(2008) Kai Y. Soo et al.   JOURNAL OF NEUROCHEMISTRY

Nrf2 Activation in Astrocytes Protects against Neurodegeneration in Mouse Models of Familial Amyotrophic Lateral Sclerosis

(2008) M. R. Vargas et al.   JOURNAL OF NEUROSCIENCE

Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis

(2008) Koji Yamanaka et al.   NATURE NEUROSCIENCE

Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice

(2008) K. Yamanaka et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Induced Pluripotent Stem Cells Generated from Patients with ALS Can Be Differentiated into Motor Neurons

(2008) J. T. Dimos et al.   SCIENCE

TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2008) J. Sreedharan et al.   SCIENCE

Timing differences of signaling response in neuron cultures activated by glutamate analogue or free radicals

(2007) Nadia Boutahar et al.   BRAIN RESEARCH

Development and Characterisation of a Glutamate-Sensitive Motor Neurone Cell Line

(2003) Christopher J. Eggett et al.   JOURNAL OF NEUROCHEMISTRY