AI SummaryThe content provided discusses a study on the effects of the mutation of the ALS-/FTD-associated RNA-binding protein FUS on axonal development. The study found that mutants of FUS, namely FUS(P525L) and FUS(16R), significantly reduced axonal complexity in vivo in a Xenopus model of ALS/FTD. Furthermore, FUS(P525L) displayed an axon looping defect in the target… Continue reading Mutation of the ALS-/FTD-Associated RNA-Binding Protein FUS Affects Axonal Development
Tag: Amyotrophic Lateral Sclerosis
New Drugs Turned Down by EU Safety Assessor
AI SummarySummary: The EU safety assessor rejected new drugs for ALS, age-related macular degeneration, and Duchenne muscular dystrophy due to insufficient evidence.Drugs for ALS, age-related macular degeneration, and Duchenne muscular dystrophy did not get the green light due to lack of evidence. Medscape Medical News
Mis-localization of endogenous TDP-43 leads to ALS-like early-stage metabolic dysfunction and progressive motor deficits
AI SummaryThis article discusses how the mis-localization of endogenous TDP-43 from the nucleus to the cytoplasm is a key factor in ALS/FTLD. It suggests that this mis-localization leads to early-stage metabolic dysfunction and progressive motor deficits similar to ALS. The study aims to better understand the role of TDP-43 in the cytoplasm and its potential… Continue reading Mis-localization of endogenous TDP-43 leads to ALS-like early-stage metabolic dysfunction and progressive motor deficits
C9ORF72 Deficiency Results in Neurodegeneration in the Zebrafish Retina
AI SummaryThe content discusses the impact of C9ORF72 deficiency in a zebrafish model, specifically focusing on neurodegeneration in the retina. The study shows that the loss of C9ORF72 function leads to various neurodegenerative features in the retina, including reduced retinal thickness, gliosis, decreased neuron levels, and disrupted outer segment structure in rod and cone cells.… Continue reading C9ORF72 Deficiency Results in Neurodegeneration in the Zebrafish Retina
Epigenomic analysis sheds light on risk factors for ALS
AI SummaryThe article discusses a study conducted by MIT researchers in collaboration with the Answer ALS consortium to analyze epigenetic modifications in motor neurons derived from 380 ALS patients. The analysis identified links between modifications and rates of disease progression, as well as genetic subtypes of ALS. The findings indicate that epigenomics may play a… Continue reading Epigenomic analysis sheds light on risk factors for ALS
Neurodegenerative disease pathways are perturbed in patients with cancer who self‐report cognitive changes and anxiety: A pathway impact analysis
AI SummaryThis study examines the pathways associated with cancer-related cognitive impairment (CRCI) and anxiety in patients undergoing chemotherapy. The analysis identifies perturbed neurodegenerative disease pathways, such as amyotrophic lateral sclerosis, Huntington disease, Parkinson disease, and prion disease. Common biological processes across these pathways include apoptosis, mitochondrial dysfunction, oxidative stress, and endoplasmic reticulum stress. The findings… Continue reading Neurodegenerative disease pathways are perturbed in patients with cancer who self‐report cognitive changes and anxiety: A pathway impact analysis
Neurodegenerative disease pathways are perturbed in patients with cancer who self‐report cognitive changes and anxiety: A pathway impact analysis
AI SummaryThe study explores the co-occurrence of cancer-related cognitive impairment (CRCI) and anxiety in cancer patients undergoing chemotherapy. Researchers identified perturbed pathways associated with these symptoms, focusing on neurodegenerative diseases like Parkinson’s and Huntingon’s disease. Common biological processes across these pathways include apoptosis, mitochondrial dysfunction, oxidative stress, and endoplasmic reticulum stress. The findings suggest potential… Continue reading Neurodegenerative disease pathways are perturbed in patients with cancer who self‐report cognitive changes and anxiety: A pathway impact analysis
Prominent ALS Researcher Moves From Treatment to Prevention
After years of disappointing ALS clinical trial results, this year’s Sheila Essey Award recipient for significant contributions in ALS research is shifting to prevention. MDedge News
Human Motor Neurons Elicit Pathological Hallmarks of ALS and Reveal Potential Biomarkers of the Disease in Response to Prolonged IFN{gamma} Exposure
AI SummaryThis article examines the impact of prolonged interferon-gamma (IFN) exposure on human motor neurons, revealing cytoplasmic aggregation of TDP-43, resulting in p53 pathway activation and neuronal dysfunction. The study suggests that IFN exposure can replicate pathological hallmarks of ALS, including altered gene expression and the potential involvement of PD-L1 in disease development. The findings… Continue reading Human Motor Neurons Elicit Pathological Hallmarks of ALS and Reveal Potential Biomarkers of the Disease in Response to Prolonged IFN{gamma} Exposure
14-3-3θ, a novel player in TDP-43 pathophysiology: Implications for ALS/FTD
AI SummaryThis article discusses a new finding about the interaction between 14-3-3θ and TDP-43 in TDP-43 proteinopathies, which are associated with ALS/FTD. The study shows that this interaction affects both loss-of-function and gain-of-toxic pathology related to TDP-43. The authors also suggest a potential gene therapy targeting 14-3-3θ to reduce deficits caused by TDP-43 in transgenic… Continue reading 14-3-3θ, a novel player in TDP-43 pathophysiology: Implications for ALS/FTD