Riluzole does not ameliorate disease caused by cytoplasmic TDP‐43 in a mouse model of amyotrophic lateral sclerosis

A new mouse line with reduced GluA2 Q/R site RNA editing exhibits loss of dendritic spines, hippocampal CA1-neuron loss, learning and memory impairments and NMDA receptor-independent seizure vulnerability

(2020) Lyndsey M. Konen et al.   Molecular Brain

Riluzole Exhibits No Therapeutic Efficacy On a Transgenic Rat model of Amyotrophic Lateral Sclerosis

(2020) Si Chen et al.   CURRENT NEUROVASCULAR RESEARCH

Riluzole improves functional recovery after acute spinal cord injury in rats and may be associated with changes in spinal microglia/macrophages polarization

(2020) Qichao Wu et al.   NEUROSCIENCE LETTERS

Amyotrophic lateral sclerosis associated mislocalisation of TDP‐43 to the cytoplasm causes cortical hyperexcitability and reduced excitatory neurotransmission in the motor cortex

(2020) MS. Dyer et al.   JOURNAL OF NEUROCHEMISTRY

Treatment with the glutamate modulator riluzole prevents early life stress-induced cognitive deficits and impairments in synaptic plasticity in APPswe/PS1dE9 mice

(2019) Sylvie L. Lesuis et al.   NEUROPHARMACOLOGY

Evidence for a multimodal effect of riluzole in patients with ALS?

(2019) Adriaan D de Jongh et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

ADAR2 mislocalization and widespread RNA editing aberrations in C9orf72-mediated ALS/FTD

(2019) Stephen Moore et al.   ACTA NEUROPATHOLOGICA

Amyotrophic lateral sclerosis mutant TDP-43 may cause synaptic dysfunction through altered dendritic spine function

(2019) Tongcui Jiang et al.   Disease Models & Mechanisms

Excess glutamate secreted from astrocytes drives upregulation of P-glycoprotein in endothelial cells in amyotrophic lateral sclerosis

(2019) Loqman A. Mohamed et al.   EXPERIMENTAL NEUROLOGY

Riluzole blocks HU210-facilitated ventral tegmental long-term depression by enhancing glutamate uptake in astrocytes

(2019) Zhiqiang Liu et al.   NEUROSCIENCE LETTERS

Tempol improves neuroinflammation and delays motor dysfunction in a mouse model (SOD1G93A) of ALS

(2019) Gabriela Bortolança Chiarotto et al.   Journal of Neuroinflammation

Dysregulation of AMPA receptor subunit expression in sporadic ALS post‐mortem brain

(2019) Jenna M Gregory et al.   JOURNAL OF PATHOLOGY

p97 regulates GluA1 homomeric AMPA receptor formation and plasma membrane expression

(2019) Yuan Ge et al.   Nature Communications

Shortened TDP43 isoforms upregulated by neuronal hyperactivity drive TDP43 pathology in ALS

(2019) Kaitlin Weskamp et al.   JOURNAL OF CLINICAL INVESTIGATION

Overexpression of human wtTDP-43 causes impairment in hippocampal plasticity and behavioral deficits in CAMKII-tTa transgenic mouse model

(2019) Zainuddin Quadri et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Nucleo-cytoplasmic transport of TDP-43 studied in real time: impaired microglia function leads to axonal spreading of TDP-43 in degenerating motor neurons

(2018) Adam J. Svahn et al.   ACTA NEUROPATHOLOGICA

Stage at which riluzole treatment prolongs survival in patients with amyotrophic lateral sclerosis: a retrospective analysis of data from a dose-ranging study

(2018) Ton Fang et al.   LANCET NEUROLOGY

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

Cell death cascade and molecular therapy in ADAR2-deficient motor neurons of ALS

(2018) Takenari Yamashita et al.   NEUROSCIENCE RESEARCH

TDP-43 self-interaction is modulated by redox-active compounds Auranofin, Chelerythrine and Riluzole

(2018) Moritz Oberstadt et al.   Scientific Reports

Hypoexcitability precedes denervation in the large fast-contracting motor units in two unrelated mouse models of ALS

(2018) María de Lourdes Martínez-Silva et al.   eLife

Targeting Protein Kinase CK1δ with Riluzole: Could It Be One of the Possible Missing Bricks to Interpret Its Effect in the Treatment of ALS from a Molecular Point of View?

(2018) Maicol Bissaro et al.   ChemMedChem

Stable transgenic C9orf72 zebrafish model key aspects of the ALS/FTD phenotype and reveal novel pathological features

(2018) Matthew P. Shaw et al.   Acta Neuropathologica Communications

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

ADAR RNA editing in human disease; more to it than meets the I

(2017) Angela Gallo et al.   HUMAN GENETICS

Acetylation-induced TDP-43 pathology is suppressed by an HSF1-dependent chaperone program

(2017) Ping Wang et al.   Nature Communications

Riluzole does not improve lifespan or motor function in three ALS mouse models

(2017) Marion C. Hogg et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Phosphorylation of TAR DNA-binding Protein of 43 kDa (TDP-43) by Truncated Casein Kinase 1δ Triggers Mislocalization and Accumulation of TDP-43

(2016) Takashi Nonaka et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Age and Alzheimer’s disease gene expression profiles reversed by the glutamate modulator riluzole

(2016) A C Pereira et al.   MOLECULAR PSYCHIATRY

Effects of riluzole on P2X7R expression in the spinal cord in rat model of neuropathic pain

(2016) Kai Jiang et al.   NEUROSCIENCE LETTERS

Riluzole exerts transient modulating effects on cortical and axonal hyperexcitability in ALS

(2016) Nimeshan Geevasinga et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Acute Footshock Stress Induces Time-Dependent Modifications of AMPA/NMDA Protein Expression and AMPA Phosphorylation

(2016) Daniela Bonini et al.   NEURAL PLASTICITY

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

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

Riluzole rescues glutamate alterations, cognitive deficits, and tau pathology associated with P301L tau expression

(2015) Holly C. Hunsberger et al.   JOURNAL OF NEUROCHEMISTRY

Autophagy in spinal motor neurons of conditional ADAR2-knockout mice: An implication for a role of calcium in increased autophagy flux in ALS

(2015) Soichi Sasaki et al.   NEUROSCIENCE LETTERS

Inhibiting drug efflux transporters improves efficacy of ALS therapeutics

(2014) Michael R. Jablonski et al.   Annals of Clinical and Translational Neurology

Novel monoclonal antibodies to normal and pathologically altered human TDP-43 proteins

(2014) Linda K Kwong et al.   Acta Neuropathologica Communications

Rescue of amyotrophic lateral sclerosis phenotype in a mouse model by intravenous AAV9-ADAR2delivery to motor neurons

(2013) Takenari Yamashita et al.   EMBO Molecular Medicine

The changing scene of amyotrophic lateral sclerosis

(2013) Wim Robberecht et al.   NATURE REVIEWS NEUROSCIENCE

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

(2013) Shuo-Chien Ling et al.   NEURON

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

Rapid Determination of Myosin Heavy Chain Expression in Rat, Mouse, and Human Skeletal Muscle Using Multicolor Immunofluorescence Analysis

(2012) Darin Bloemberg et al.   PLoS One

Identification of RNA bound to the TDP-43 ribonucleoprotein complex in the adult mouse brain

(2012) Ramesh K. Narayanan et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

The essential role of AMPA receptor GluR2 subunit RNA editing in the normal and diseased brain

(2012) Amanda Wright et al.   Frontiers in Molecular Neuroscience

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

(2011) Magdalini Polymenidou et al.   NATURE NEUROSCIENCE

Characterizing the RNA targets and position-dependent splicing regulation by TDP-43

(2011) James R Tollervey et al.   NATURE NEUROSCIENCE

Riluzole elevates GLT-1 activity and levels in striatal astrocytes

(2011) Marica Carbone et al.   NEUROCHEMISTRY INTERNATIONAL

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

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

A Review of the Neural Mechanisms of Action and Clinical Efficiency of Riluzole in Treating Amyotrophic Lateral Sclerosis: What have we Learned in the Last Decade?

(2010) Mark C. Bellingham   CNS Neuroscience & Therapeutics

Effects of gender in amyotrophic lateral sclerosis

(2010) Pamela A. McCombe et al.   Gender Medicine

Identification of Neuronal RNA Targets of TDP-43-containing Ribonucleoprotein Complexes

(2010) Chantelle F. Sephton et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Enhanced endogenous type I interferon cell-driven survival and inhibition of spontaneous apoptosis by Riluzole

(2009) Ammar Achour et al.   VIROLOGY

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

(2008) Sean Scott et al.   Amyotrophic Lateral Sclerosis

Riluzole blocks persistent Na+and Ca2+currents and modulates release of glutamate via presynaptic NMDA receptors on neonatal rat hypoglossal motoneuronsin vitro

(2008) Nerijus Lamanauskas et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Preferential motor unit loss in the SOD1G93Atransgenic mouse model of amyotrophic lateral sclerosis

(2008) J. Hegedus et al.   JOURNAL OF PHYSIOLOGY-LONDON

Riluzole enhances the activity of glutamate transporters GLAST, GLT1 and EAAC1

(2007) Elena Fumagalli et al.   EUROPEAN JOURNAL OF PHARMACOLOGY