Loss of function of C9orf72 causes motor deficits in a zebrafish model of amyotrophic lateral sclerosis

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Items 101–200 of 334

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4. Expression of C9orf72-related dipeptides impairs motor function in a vertebrate model
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5. C9orf72 plays a central role in Rab GTPase-dependent regulation of autophagy
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6. Knocking out C9ORF72 Exacerbates Axonal Trafficking Defects Associated with Hexanucleotide Repeat Expansion and Reduces Levels of Heat Shock Proteins
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8. Fishing for causes and cures of motor neuron disorders
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9. The Enigmatic Role of C9ORF72 in Autophagy
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10. Targeting phosphoglycerate kinase 1 with terazosin improves motor neuron phenotypes in multiple models of amyotrophic lateral sclerosis
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11. Multifaceted Genes in Amyotrophic Lateral Sclerosis-Frontotemporal Dementia
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12. RNA-mediated toxicity in C9orf72 ALS and FTD
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13. Genetic models of C9orf72: what is toxic?
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14. R-Loops in Motor Neuron Diseases
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15. Artificial MicroRNAs Targeting C9orf72 Can Reduce Accumulation of Intra-nuclear Transcripts in ALS and FTD Patients
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16. IN VITRO AND IN VIVO MODELS OF AMYOTROPHIC LATERAL SCLEROSIS: AN UPDATED OVERVIEW
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17. Antisense Oligonucleotide Therapy for the Treatment of C9ORF72 ALS/FTD Diseases
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19. Worming forward: amyotrophic lateral sclerosis toxicity mechanisms and genetic interactions in Caenorhabditis elegans
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20. Molecular mechanisms and animal models of spinal muscular atrophy
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21. Drosophila Glia: Models for Human Neurodevelopmental and Neurodegenerative Disorders
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22. Modeling Environmentally-Induced Motor Neuron Degeneration in Zebrafish
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23. Modelling C9ORF72 hexanucleotide repeat expansion in amyotrophic lateral sclerosis and frontotemporal dementia
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24. C9orf72 associates with inactive Rag GTPases and regulates mTORC1‐mediated autophagosomal and lysosomal biogenesis
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27. Expression of ALS/FTD-linked mutant CCNF in zebrafish leads to increased cell death in the spinal cord and an aberrant motor phenotype
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28. A novel ALS-associated variant in UBQLN4 regulates motor axon morphogenesis
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29. Synaptic localization of C9orf72 regulates post-synaptic glutamate receptor 1 levels
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30. Unaffected mosaic C9orf72 case
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31. Diagnostic Challenge and Neuromuscular Junction Contribution to ALS Pathogenesis
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32. Synaptic dysfunction induced by glycine‐alanine dipeptides in C9orf72‐ALS/FTD is rescued by SV2 replenishment
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33. C9orf72 isoforms in Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration
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34. Invited Review: Decoding the pathophysiological mechanisms that underlie RNA dysregulation in neurodegenerative disorders: a review of the current state of the art
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35. INaP selective inhibition reverts precocious inter- and motorneurons hyperexcitability in the Sod1-G93R zebrafish ALS model
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36. Pathogenic determinants and mechanisms of ALS/FTD linked to hexanucleotide repeat expansions in the C9orf72 gene
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37. C9orf72 is essential for neurodevelopment and motility mediated by Cyclin G1
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38. RNA Binding Proteins and the Pathogenesis of Frontotemporal Lobar Degeneration
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39. The Genetics of C9orf72 Expansions
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40. Molecular Network Analysis Suggests a Logical Hypothesis for the Pathological Role of C9orf72 in Amyotrophic Lateral Sclerosis/Frontotemporal Dementia
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41. Spatial transcriptomics identifies spatially dysregulated expression of GRM3 and USP47 in amyotrophic lateral sclerosis
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42. Modelling amyotrophic lateral sclerosis in rodents
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43. C9ORF72 expression and cellular localization over mouse development
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44. Arginine-rich dipeptide-repeat proteins as phase disruptors in C9-ALS/FTD
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45. ALS-related misfolded protein management in motor neurons and muscle cells
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46. Pathogenic Mechanisms and Therapy Development for C9orf72 Amyotrophic Lateral Sclerosis/Frontotemporal Dementia
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47. The Development of C9orf72-Related Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Disorders
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48. The C9ORF72 Gene, Implicated in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia, Encodes a Protein That Functions in Control of Endothelin and Glutamate Signaling
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49. Functional Characterisation of a GWAS Risk Locus Identifies GPX3 as a Lead Candidate Gene in ALS
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50. A 30-unit hexanucleotide repeat expansion in C9orf72 induces pathological lesions with dipeptide-repeat proteins and RNA foci, but not TDP-43 inclusions and clinical disease
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51. BMAA and MCLR Interact to Modulate Behavior and Exacerbate Molecular Changes Related to Neurodegeneration in Larval Zebrafish
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52. Development of Therapeutics for C9ORF72 ALS/FTD-Related Disorders
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53. Stratified gene expression analysis identifies major amyotrophic lateral sclerosis genes
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54. G-quadruplex poses quadruple threat
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55. C9orf72’s Interaction with Rab GTPases—Modulation of Membrane Traffic and Autophagy
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56. Amyotrophic Lateral Sclerosis Genes in Drosophila melanogaster
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57. Where and Why Modeling Amyotrophic Lateral Sclerosis
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58. Expression of ALS/FTD-linked mutant CCNF in zebrafish leads to increased cell death in the spinal cord and an aberrant motor phenotype
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59. Crosstalk between Different DNA Repair Pathways Contributes to Neurodegenerative Diseases
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60. Biomarker development for C9orf72 repeat expansion in ALS
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61. Current developments in gene therapy for amyotrophic lateral sclerosis
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62. New Antibody-Free Mass Spectrometry-Based Quantification Reveals That C9ORF72 Long Protein Isoform Is Reduced in the Frontal Cortex of Hexanucleotide-Repeat Expansion Carriers
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65. Divergence, Convergence, and Therapeutic Implications: A Cell Biology Perspective of C9ORF72-ALS/FTD
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66. Bromodomain inhibitors regulate the C9ORF72 locus in ALS
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68. Efficacy of Ciprofloxacin/Celecoxib combination in zebrafish models of amyotrophic lateral sclerosis
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69. Glial Cell Dysfunction in C9orf72-Related Amyotrophic Lateral Sclerosis and Frontotemporal Dementia
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70. Emerging role of RNA•DNA hybrids inC9orf72-linked neurodegeneration
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72. TDP-43 Regulation of AChE Expression Can Mediate ALS-Like Phenotype in Zebrafish
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73. Microglial crosstalk with astrocytes and immune cells in amyotrophic lateral sclerosis
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74. Amyotrophic Lateral Sclerosis Pathogenesis Converges on Defects in Protein Homeostasis Associated with TDP-43 Mislocalization and Proteasome-Mediated Degradation Overload
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75. C9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten
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76. La accesibilidad de los portales web de las universidades públicas andaluzas
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77. RNA-Targeted Therapies and Amyotrophic Lateral Sclerosis
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78. Effects of intracellular calcium accumulation on proteins encoded by the major genes underlying amyotrophic lateral sclerosis
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80. Mechanisms of repeat-associated non-AUG translation in neurological microsatellite expansion disorders
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81. Poly-GP accumulation due to C9orf72 loss of function induces motor neuron apoptosis through autophagy and mitophagy defects
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83. Neural Differentiation and spinal cord organoid generation from induced pluripotent stem cells (iPSCs) for ALS modelling and inflammatory screening
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84. Combined Transcriptomics and Proteomics in Frontal Cortex Area 8 in Frontotemporal Lobar Degeneration Linked to C9ORF72 Expansion
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85. Acrolein-inducing ferroptosis contributes to impaired peripheral neurogenesis in zebrafish
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90. Novel genome-editing-based approaches to treat motor neuron diseases: Promises and challenges
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93. C9orf72-associated SMCR8 protein binds in the ubiquitin pathway and with proteins linked with neurological disease
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95. Repeat-associated non-AUG (RAN) translation mechanisms are running into focus for GGGGCC-repeat associated ALS/FTD
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100. RNA Is a Double-Edged Sword in ALS Pathogenesis
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