Extreme Exercisers May Have Higher Odds for ALS

MONDAY, April 23, 2018 -- Fitness buffs who push themselves to the limit during workouts might slightly increase their risk of developing amyotrophic lateral sclerosis (ALS), a new study suggests. The same may hold true for working stiffs whose jobs...
Source: Drugs.com - Daily MedNews - Category: General Medicine Source Type: news

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Doroth ée Lulé, Andrea Kübler, Albert C. Ludolph
Source: Frontiers in Neurology - Category: Neurology Source Type: research
This study examined the levels of neuroinflammatory proteins in the spinal cord of JGT-treated hSOD1 G93A transgenic mice to determine the effect of Jaeumganghwa-Tang (JGT) on neuroinflammation. Twelve 8-week-old male experimental mice were randomly allocated to three groups: a non-transgenic group, a hSOD1G93A transgenic group, and a hSOD1G93A transgenic group that received JGT 1 mg/g orally once daily for 6 weeks. After 6 weeks, the spinal cord tissues were analyzed for inflammatory proteins (Iba-1, toll-like receptor 4, and tumor necrosis factor-α) and oxidative stress-related proteins (transferrin, ferriti...
Source: Evidence-based Complementary and Alternative Medicine - Category: Complementary Medicine Tags: Evid Based Complement Alternat Med Source Type: research
DiscussionAs this SNP is known to modify the detoxifying activity of paraxonase 1 with respect to different substrates as well as other activities of the protein, we hypothesize that the identified association might reflect specific motor neuron vulnerability to certain exogenous toxic substances metabolized less efficiently by the 192R alloenzyme, or to detrimental endogenous pathophysiological processes such as oxidative stress. Further exploration of this possible metabolic susceptibility could deepen our knowledge of ALS pathomechanisms.
Source: Neurological Sciences - Category: Neurology Source Type: research
In recent years researchers have investigated changes in alternative splicing in the context of aging and age-related disease. It is thought to be important in cellular senescence, for example, but that is just one line item in the bigger picture. A given gene can code for multiple different proteins, and alternative splicing is the name given to the processes by which those different proteins are produced. A gene contains discrete DNA sequences called exons and introns, the former passed into the protein production process, and the latter removed during RNA splicing. The canonical protein produced from this genetic bluepr...
Source: Fight Aging! - Category: Research Authors: Tags: Medicine, Biotech, Research Source Type: blogs
Publication date: Available online 21 March 2019Source: Journal of Molecular BiologyAuthor(s): Sei-Kyoung Park, Sangeun Park, Susan W. LiebmanAbstractThe trans-activating response DNA-binding protein 43 (TDP-43) is a transcriptional repressor and splicing factor. TDP-43 is normally mostly in the nucleus, although it shuttles to the cytoplasm. Mutations in TDP-43 are one cause of familial amyotrophic lateral sclerosis (ALS). In neurons of these patients, TDP-43 forms cytoplasmic aggregates. In addition, wild-type TDP-43 is also frequently found in neuronal cytoplasmic aggregates in patients with neurodegenerative diseases n...
Source: Journal of Molecular Biology - Category: Molecular Biology Source Type: research
The aim of our study was to evaluate the role of mutations in theMAPT gene in patients with pure amyotrophic lateral sclerosis (ALS). A cohort of 120 ALS patients, both sporadic and familial, without cognitive impairment was analyzed by next-generation sequencing with a multiple-gene panel comprising 23 genes, includingMAPT, known to be associated with ALS and frontotemporal dementia. The presence of theC9orf72 expansion was also investigated. Twelve patients had mutations in theSOD1, TARDBP, MATR3, andFUS genes, while 10 patients carried theC9orf72 expansion. One female patient was found to carry the D348G mutation inMAPT...
Source: Neurodegenerative Diseases - Category: Neurology Source Type: research
Stress granules (SGs) are non-membrane-bound RNA-protein granules that assemble through phase separation in response to cellular stress. Disturbances in SG dynamics have been implicated as a primary driver of neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), suggesting the hypothesis that these diseases reflect an underlying disturbance in the dynamics and material properties of SGs. However, this concept has remained largely untestable in available models of SG assembly, which require the confounding variable of exogenous stressors. Here we introduce a light-induc...
Source: eLife - Category: Biomedical Science Tags: Cell Biology Source Type: research
Abstract The aim of our study was to evaluate the role of mutations in the MAPT gene in patients with pure amyotrophic lateral sclerosis (ALS). A cohort of 120 ALS patients, both sporadic and familial, without cognitive impairment was analyzed by next-generation sequencing with a multiple-gene panel comprising 23 genes, including MAPT, known to be associated with ALS and frontotemporal dementia. The presence of the C9orf72 expansion was also investigated. Twelve patients had mutations in the SOD1, TARDBP, MATR3, and FUS genes, while 10 patients carried the C9orf72 expansion. One female patient was found to carry t...
Source: Neuro-Degenerative Diseases - Category: Neurology Authors: Tags: Neurodegener Dis Source Type: research
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Source: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration - Category: Neurology Authors: Source Type: research
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Source: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration - Category: Neurology Authors: Source Type: research
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