NLRP3 Inflammasome Activation in a Transgenic Amyotrophic Lateral Sclerosis Model

In conclusion, our results showed the activation of the NLRP3 inflammasome in the brain of SOD1G93A rats, indicating that inflammation plays a main role in ALS.
Source: Inflammation - Category: Allergy & Immunology Source Type: research

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Abstract Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by progressive degeneration and loss of motor neurons that appears to spread through the neuroaxis in a spatiotemporally restricted manner. In the familial form of ALS, the presence of any one of over 180 inherited mutations in the gene that encodes Cu/Zn superoxide dismutase (SOD1) leads to its eventual misfolding and aggregation. Once the pathological SOD1 seed is formed, it can continue growing into a larger aggregate through nucleation of other SOD1 substrate molecules. To date, there is no effective and rapid metho...
Source: Mol Biol Cell - Category: Molecular Biology Authors: Tags: Methods Mol Biol Source Type: research
Publication date: Available online 20 October 2018Source: Biochimica et Biophysica Acta (BBA) - Molecular Basis of DiseaseAuthor(s): Samantha N. Cobos, Seth A. Bennett, Mariana P. TorrenteAbstractEvery year, neurodegenerative disorders take more than 5000 lives in the US alone. Cures have not yet been found for many of the multitude of neuropathies. The majority of amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD) and Parkinson's disease (PD) cases have no known genetic basis. Thus, it is evident that contemporary genetic approaches have failed to explain the etiology or etiologies of ALS/FTD and PD. Recen...
Source: Biochimica et Biophysica Acta (BBA) Molecular Basis of Disease - Category: Molecular Biology Source Type: research
Contributors : Kentaro Otake ; Hidenori Kamiguchi ; Yoshihiko HirozaneSeries Type : Expression profiling by high throughput sequencingOrganism : Homo sapiensIn comparison with exosomal mRNAs in CSF from four patients with amyotrophic lateral sclerosis, 543 genes were significantly changed, as represented by CUEDC2.
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Homo sapiens Source Type: research
Assessing the Role of Recent Therapeutic Breakthroughs in the Treatment of Amyotrophic Lateral Sclerosis
Source: Peerview CME/CE Audio Podcast - Neurology International - Category: Neurology Authors: Tags: Science, Medicine Source Type: podcasts
Objective The aim of the study was to systematize the disability condition related to amyotrophic lateral sclerosis patients using the International Classification of Functioning, Disability and Health (ICF) and narrative medicine approach as a common tool to identify a patient's functional problems. Once identified, this can be used as the basis for an individual rehabilitation project. Design This is an observational study on patients residing in a central region of Italy with a diagnosis of amyotrophic lateral sclerosis. The narrative approach involved listening to the patients' stories while guiding them with a se...
Source: American Journal of Physical Medicine and Rehabilitation - Category: Rehabilitation Tags: Original Research Articles Source Type: research
Brain and Behavior, EarlyView.
Source: Brain and Behavior - Category: Neurology Authors: Source Type: research
Muscle&Nerve, EarlyView.
Source: Muscle and Nerve - Category: Internal Medicine Authors: Source Type: research
Microsatellite expansions cause more than 40 neurological disorders, including Huntington's disease, myotonic dystrophy, and C9ORF72 amyotrophic lateral sclerosis/frontotemporal dementia (ALS/FTD). These repeat expansion mutations can produce repeat-associated non-ATG (RAN) proteins in all three reading frames, which accumulate in disease-relevant tissues. There has been considerable interest in RAN protein products and their downstream consequences, particularly for the dipeptide proteins found in C9ORF72 ALS/FTD. Understanding how RAN translation occurs, what cellular factors contribute to RAN protein accumulation, and h...
Source: Journal of Biological Chemistry - Category: Chemistry Authors: Tags: Minireviews Source Type: research
Publication date: Available online 16 October 2018Source: Journal of Biomedical InformaticsAuthor(s): M. Ahangaran, M.R. Jahed-Motlagh, B. Minaei-BidgoliAbstractOne of the most important issues in predictive modeling is to determine major cause factors of a phenomenon and causal relationships between them. Extracting causal relationships between parameters in a natural phenomenon can be accomplished through checking the parameters’ changes in consecutive events. In addition, using information and probabilistic theory help better conception of causal relationships of a phenomenon. Therefore, probabilistic causal disco...
Source: Journal of Biomedical Informatics - Category: Information Technology Source Type: research
Authors: Ohnari K, Okada K, Higuchi O, Matsuo H, Adachi H Abstract An 82-year-old woman developed neck weakness and dysarthria with antibodies against acetylcholine receptor (AChR) and low-density lipoprotein receptor-related protein 4 (LRP4). Myasthenia gravis (MG) was diagnosed by edrophonium and repetitive nerve stimulation tests. Her symptoms resolved completely by immunotherapy. One year later, she presented with muscle weakness and bulbar palsy accompanied by atrophy and fasciculation. Her tendon reflexes were brisk, and Babinski's sign was positive. She was diagnosed with probable amyotrophic lateral scleros...
Source: Internal Medicine - Category: Internal Medicine Tags: Intern Med Source Type: research
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