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Cellular Chaperones As Therapeutic Targets in ALS to Restore Protein Homeostasis and Improve Cellular Function.
- Published in:
- Frontiers in Molecular Neuroscience, 2017, p. 1, doi. 10.3389/fnmol.2017.00251
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- Publication type:
- Article
Unexpected frequency of the pathogenic AR CAG repeat expansion in the general population.
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- Brain: A Journal of Neurology, 2023, v. 146, n. 7, p. 2723, doi. 10.1093/brain/awad050
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- Publication type:
- Article
Astrocytes display cell autonomous and diverse early reactive states in familial amyotrophic lateral sclerosis.
- Published in:
- 2022
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- Publication type:
- journal article
Widespread FUS mislocalization is a molecular hallmark of amyotrophic lateral sclerosis.
- Published in:
- Brain: A Journal of Neurology, 2019, v. 142, n. 9, p. 2572, doi. 10.1093/brain/awz217
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- Publication type:
- Article
Widespread FUS mislocalization is a molecular hallmark of amyotrophic lateral sclerosis.
- Published in:
- 2019
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- Publication type:
- journal article
Humanized mutant FUS drives progressive motor neuron degeneration without aggregation in 'FUSDelta14' knockin mice.
- Published in:
- 2017
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- Publication type:
- journal article
Enhanced Expression of TRAP1 Protects Mitochondrial Function in Motor Neurons under Conditions of Oxidative Stress.
- Published in:
- International Journal of Molecular Sciences, 2022, v. 23, n. 3, p. 1789, doi. 10.3390/ijms23031789
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- Publication type:
- Article
Activation of the heat shock response in a primary cellular model of motoneuron neurodegeneration-evidence for neuroprotective and neurotoxic effects.
- Published in:
- Cellular & Molecular Biology Letters, 2009, v. 14, n. 2, p. 319, doi. 10.2478/s11658-009-0002-8
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- Publication type:
- Article
Creating Interactions between Tissue-Engineered Skeletal Muscle and the Peripheral Nervous System.
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- Cells Tissues Organs, 2016, v. 202, n. 3/4, p. 143, doi. 10.1159/000443634
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- Publication type:
- Article
Microglial Expression of theWnt Signaling Modulator DKK2 Differs between Human Alzheimer's Disease Brains andMouse Neurodegeneration Models.
- Published in:
- eNeuro, 2023, v. 10, n. 1, p. 1, doi. 10.1523/ENEURO.0306-22.2022
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- Publication type:
- Article
Silencing mutant SOD1 using RNAi protects against neurodegeneration and extends survival in an ALS model.
- Published in:
- Nature Medicine, 2005, v. 11, n. 4, p. 429, doi. 10.1038/nm1205
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- Publication type:
- Article
Treatment with arimoclomol, a coinducer of heat shock proteins, delays disease progression in ALS mice.
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- Nature Medicine, 2004, v. 10, n. 4, p. 402, doi. 10.1038/nm1021
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- Article
Analysis of motor dysfunction in Down Syndrome reveals motor neuron degeneration.
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- 2018
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- Publication type:
- Abstract
The Role of the Protein Quality Control System in SBMA.
- Published in:
- Journal of Molecular Neuroscience, 2016, v. 58, n. 3, p. 348, doi. 10.1007/s12031-015-0675-6
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- Publication type:
- Article
Targeted Molecular Therapies for SBMA.
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- Journal of Molecular Neuroscience, 2016, v. 58, n. 3, p. 335, doi. 10.1007/s12031-015-0676-5
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- Publication type:
- Article
A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice.
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- Journal of Cell Biology, 2005, v. 169, n. 4, p. 561, doi. 10.1083/jcb.200501085
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- Publication type:
- Article
A Nonsense Mutation in Mouse <i>Tardbp</i> Affects TDP43 Alternative Splicing Activity and Causes Limb-Clasping and Body Tone Defects.
- Published in:
- PLoS ONE, 2014, v. 9, n. 1, p. 1, doi. 10.1371/journal.pone.0085962
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- Publication type:
- Article
Molecular Chaperone Mediated Late-Stage Neuroprotection in the SOD1<sup>G93A</sup> Mouse Model of Amyotrophic Lateral Sclerosis.
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- PLoS ONE, 2013, v. 8, n. 8, p. 1, doi. 10.1371/journal.pone.0073944
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- Publication type:
- Article
Plasma Neurofilament Heavy Chain Levels Correlate to Markers of Late Stage Disease Progression and Treatment Response in SOD1<sup>G93A</sup> Mice that Model ALS.
- Published in:
- PLoS ONE, 2012, v. 7, n. 7, p. 1, doi. 10.1371/journal.pone.0040998
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- Publication type:
- Article
Modification of Superoxide Dismutase 1 (SOD1) Properties by a GFP Tag - Implications for Research into Amyotrophic Lateral Sclerosis (ALS).
- Published in:
- PLoS ONE, 2010, v. 5, n. 3, p. 1, doi. 10.1371/journal.pone.0009541
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- Publication type:
- Article
Mutant Glycyl-tRNA Synthetase (Gars) Ameliorates SOD1<sup>G93A</sup> Motor Neuron Degeneration Phenotype but Has Little Affect on Loa Dynein Heavy Chain Mutant Mice.
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- PLoS ONE, 2009, v. 4, n. 7, p. 1, doi. 10.1371/journal.pone.0006218
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- Publication type:
- Article
Plasma neurofilament heavy chain is not a useful biomarker in Charcot-Marie-Tooth disease.
- Published in:
- 2016
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- Publication type:
- journal article
Excitability properties of mouse motor axons in the mutant SOD1(G93A) model of amyotrophic lateral sclerosis.
- Published in:
- 2010
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- Publication type:
- journal article
Excitability properties of mouse motor axons in the mutant SOD1<sup>G93A</sup> model of amyotrophic lateral sclerosis.
- Published in:
- Muscle & Nerve, 2010, v. 41, n. 6, p. 774, doi. 10.1002/mus.21579
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- Publication type:
- Article
IGF1R regulates retrograde axonal transport of signalling endosomes in motor neurons.
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- EMBO Reports, 2020, v. 21, n. 3, p. 1, doi. 10.15252/embr.201949129
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- Publication type:
- Article
An optogenetic cell therapy to restore control of target muscles in an aggressive mouse model of amyotrophic lateral sclerosis.
- Published in:
- eLife, 2024, p. 1, doi. 10.7554/eLife.88250
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- Publication type:
- Article
Expression of Kv3.1b potassium channel is widespread in macaque motor cortex pyramidal cells: A histological comparison between rat and macaque.
- Published in:
- Journal of Comparative Neurology, 2017, v. 525, n. 9, p. 2164, doi. 10.1002/cne.24192
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- Publication type:
- Article
Endoplasmic reticulum stress in spinal and bulbar muscular atrophy: a potential target for therapy.
- Published in:
- Brain: A Journal of Neurology, 2014, v. 137, n. 7, p. 1894, doi. 10.1093/brain/awu114
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- Publication type:
- Article
Co-induction of the heat shock response ameliorates disease progression in a mouse model of human spinal and bulbar muscular atrophy: implications for therapy.
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- Brain: A Journal of Neurology, 2013, v. 136, n. 3, p. 926, doi. 10.1093/brain/aws343
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- Publication type:
- Article
The altered expression of neurofilament in mouse models and patients with spinal muscular atrophy.
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- Annals of Clinical & Translational Neurology, 2021, v. 8, n. 4, p. 866, doi. 10.1002/acn3.51336
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- Publication type:
- Article
Automated and unbiased discrimination of ALS from control tissue at single cell resolution.
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- Brain Pathology, 2021, v. 31, n. 4, p. 1, doi. 10.1111/bpa.12937
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- Publication type:
- Article
Increasing cannabinoid levels by pharmacological and genetic manipulation delays disease progression in SOD1 mice.
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- FASEB Journal, 2006, v. 20, n. 7, p. 1003, doi. 10.1096/fj.05-4743fje
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- Publication type:
- Article
A motor neuron strategy to save time and energy in neurodegeneration: adaptive protein stoichiometry.
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- Journal of Neurochemistry, 2018, v. 146, n. 5, p. 631, doi. 10.1111/jnc.14542
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- Publication type:
- Article
Activated leukocyte cell adhesion molecule modulates neurotrophin signaling.
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- Journal of Neurochemistry, 2012, v. 121, n. 4, p. 575, doi. 10.1111/j.1471-4159.2012.07658.x
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- Publication type:
- Article
Mitochondrial deficits and abnormal mitochondrial retrograde axonal transport play a role in the pathogenesis of mutant Hsp27-induced Charcot Marie Tooth Disease.
- Published in:
- Human Molecular Genetics, 2017, v. 26, n. 17, p. 3313, doi. 10.1093/hmg/ddx216
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- Publication type:
- Article
Expression of a pathogenic mutation of SOD1 sensitizes aprataxin-deficient cells and mice to oxidative stress and triggers hallmarks of premature ageing
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- Human Molecular Genetics, 2015, v. 24, n. 3, p. 828, doi. 10.1093/hmg/ddu500
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- Publication type:
- Article
Treatment with an antibody directed against Nogo-A delays disease progression in the SOD1G93A mouse model of Amyotrophic lateral sclerosis.
- Published in:
- Human Molecular Genetics, 2014, v. 23, n. 16, p. 4187, doi. 10.1093/hmg/ddu136
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- Publication type:
- Article
Amyloid precursor protein (APP) contributes to pathology in the SOD1G93A mouse model of amyotrophic lateral sclerosis.
- Published in:
- Human Molecular Genetics, 2012, v. 21, n. 17, p. 3871, doi. 10.1093/hmg/dds215
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- Publication type:
- Article
Absence of disturbed axonal transport in spinal and bulbar muscular atrophy.
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- Human Molecular Genetics, 2011, v. 20, n. 9, p. 1776, doi. 10.1093/hmg/ddr061
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- Publication type:
- Article
Inhibiting p38 MAPK alpha rescues axonal retrograde transport defects in a mouse model of ALS.
- Published in:
- Cell Death & Disease, 2018, v. 9, n. 6, p. 1, doi. 10.1038/s41419-018-0624-8
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- Publication type:
- Article
AR cooperates with SMAD4 to maintain skeletal muscle homeostasis.
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- Acta Neuropathologica, 2022, v. 143, n. 6, p. 713, doi. 10.1007/s00401-022-02428-1
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- Publication type:
- Article
Senataxin mutations elicit motor neuron degeneration phenotypes and yield TDP-43 mislocalization in ALS4 mice and human patients.
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- Acta Neuropathologica, 2018, v. 136, n. 3, p. 425, doi. 10.1007/s00401-018-1852-9
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- Publication type:
- Article
Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion.
- Published in:
- Acta Neuropathologica, 2013, v. 125, n. 2, p. 273, doi. 10.1007/s00401-012-1043-z
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- Publication type:
- Article
Regional Differences in Heat Shock Protein 25 Expression in Brain and Spinal Cord Astrocytes of Wild-Type and SOD1 G93A Mice.
- Published in:
- Cells (2073-4409), 2021, v. 10, n. 5, p. 1257, doi. 10.3390/cells10051257
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- Publication type:
- Article
Intron retention and nuclear loss of SFPQ are molecular hallmarks of ALS.
- Published in:
- Nature Communications, 2018, v. 9, n. 1, p. 1, doi. 10.1038/s41467-018-04373-8
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- Publication type:
- Article
Ongoing Developments in Sporadic Inclusion Body Myositis.
- Published in:
- Current Rheumatology Reports, 2014, v. 16, n. 12, p. 1, doi. 10.1007/s11926-014-0477-9
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- Publication type:
- Article
HDAC4-Myogenin Axis As an Important Marker of HD-Related Skeletal Muscle Atrophy.
- Published in:
- PLoS Genetics, 2015, v. 11, n. 3, p. 1, doi. 10.1371/journal.pgen.1005021
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- Publication type:
- Article
Gene expression analysis reveals early dysregulation of disease pathways and links Chmp7 to pathogenesis of spinal and bulbar muscular atrophy.
- Published in:
- Scientific Reports, 2019, v. 9, n. 1, p. 1, doi. 10.1038/s41598-019-40118-3
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- Publication type:
- Article
Upregulation of PKD1L2 provokes a complex neuromuscular disease in the mouse.
- Published in:
- Human Molecular Genetics, 2009, v. 18, n. 19, p. 3553, doi. 10.1093/hmg/ddp304
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- Publication type:
- Article
Opinion: more mouse models and more translation needed for ALS.
- Published in:
- Molecular Neurodegeneration, 2023, v. 18, n. 1, p. 1, doi. 10.1186/s13024-023-00619-2
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- Publication type:
- Article