Molecular culprits of protein aggregation in ALS and FTLD

(VIB (the Flanders Institute for Biotechnology)) The mutated and aggregated protein FUS is implicated in two neurodegenerative diseases: amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). Using a newly developed fruit fly model, researchers led by prof. Ludo Van Den Bosch (VIB-KU Leuven) have zoomed in on the protein structure of FUS to gain more insight into how it causes neuronal toxicity and disease.
Source: EurekAlert! - Medicine and Health - Category: International Medicine & Public Health Source Type: news

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The prevalence data presented in this report aims to further our understanding of the epidemiology of ALS cases in the United States.Morbidity &Mortality Weekly Report
Source: Medscape Today Headlines - Category: Consumer Health News Tags: Neurology & Neurosurgery Journal Article Source Type: news
Amyotrophic Lateral Sclerosis (ALS) is a rapid progressive neurodegenerative disease, characterized by a selective loss of motor neurons, brain stem and spinal cord which leads to deterioration of motor abilit...
Source: BMC Neurology - Category: Neurology Authors: Tags: Research article Source Type: research
ConclusionsThe present work is the first in vivo FDG-PET study showing the heterogeneous patterns of brain regional hypo- and hypermetabolism in single patients sharing C9orf72 mutation. Brain hypometabolism was consistent with the clinical phenotypes, supporting the diagnostic importance of neuroimaging functional biomarkers to capture at single-subject level specific brain dysfunction.
Source: Neurological Sciences - Category: Neurology Source Type: research
Publication date: Available online 13 December 2018Source: The Lancet NeurologyAuthor(s): Nilo Riva, Gabriele Mora, Gianni Sorarù, Christian Lunetta, Ottavia E Ferraro, Yuri Falzone, Letizia Leocani, Raffaella Fazio, Mauro Comola, Giancarlo Comi, Fabio Formaglio, Paolo Rossi, Marta Clerici, Yuri Matteo Falzone, Laura Pozzi, Daniele Martinelli, Federica Cerri, Ignazio Diego Lopez, Filippo Martinelli-Boneschi, Angelo QuattriniSummaryBackgroundSpasticity is a major determinant of disability and decline in quality of life in patients with motor neuron disease. Cannabinoids have been approved for symptomatic treatment of...
Source: The Lancet Neurology - Category: Neurology Source Type: research
Authors: Kim SJ, Park YC, Baek YH, Seo BK Abstract Wilting disorder is an abnormal condition characterized by weakness and paralysis of the upper and lower extremities. Pathogenesis and treatment target of the disorder are unclear; hence, allopathic treatment is generally used to relieve the symptoms. To investigate the treatment mechanism and effect of Traditional Korean Medicine (TKM) in patients with wilting disorder, we reviewed in vivo studies that focused on the effect of TKM on the main symptoms of wilting disorder and treatment of the diseases that can cause these symptoms. We electronically searched the Pu...
Source: Evidence-based Complementary and Alternative Medicine - Category: Complementary Medicine Tags: Evid Based Complement Alternat Med Source Type: research
This study aimed to explore the monitoring value of multimodal magnetic resonance imaging (MRI) in the disease progression of ALS. Methods: From September 2015 to March 2017, ten patients diagnosed with ALS in Peking Union Medical College Hospital completed head MRI scans at baseline and during follow-up. Multimodal MRI analyses, including gray matter (GM) volume measured by voxel-based morphometry; cerebral blood flow (CBF) evaluated by arterial spin labeling; functional connectivity, including low-frequency fluctuation (fALFF) and regional homogeneity (ReHo), measured by resting-state functional MRI; and integrity o...
Source: Chinese Medical Journal - Category: General Medicine Authors: Tags: Chin Med J (Engl) Source Type: research
To assess the feasibility of using automated capture of Electronic Medical Record (EMR) data to build predictive models for amyotrophic lateral sclerosis (ALS) outcomes.
Source: BMC Neurology - Category: Neurology Authors: Tags: Research article Source Type: research
In conclusion, this study found no evidence that mercury exposure from eating seafood, or from mercury dental fillings, was associated with the risk of developing ALS. Therefore, if mercury does play a role in the pathogenesis of ALS, other sources of exposure to mercury in the environment or workplace need to be considered. Alternatively, a susceptibility to mercury toxicity in ALS, such as genetic or epigenetic variations, multiple toxic metal interactions, or selenium deficiency, may be present.
Source: International Journal of Environmental Research and Public Health - Category: Environmental Health Authors: Tags: Article Source Type: research
In conclusion, this study found no evidence that mercury exposure from eating seafood, or from mercury dental fillings, was associated with the risk of developing ALS. Therefore, if mercury does play a role in the pathogenesis of ALS, other sources of exposure to mercury in the environment or workplace need to be considered. Alternatively, a susceptibility to mercury toxicity in ALS, such as genetic or epigenetic variations, multiple toxic metal interactions, or selenium deficiency, may be present.
Source: International Journal of Environmental Research and Public Health - Category: Environmental Health Authors: Tags: Article Source Type: research
Human pluripotent stem cells hold great promise for improving regenerative medicine. However, a risk for tumor formation and difficulties in generating large amounts of subtype derivatives remain the major obstacles for clinical applications of stem cells. Here, we discovered that zinc finger E-box–binding homeobox 1 (ZEB1) is highly expressed upon differentiation of human embryonic stem cells (hESCs) into neuronal precursors. CRISPR/Cas9-mediated ZEB1 depletion did not impede neural fate commitment, but prevented hESC-derived neural precursors from differentiating into neurons, indicating that ZEB1 is required for n...
Source: Journal of Biological Chemistry - Category: Chemistry Authors: Tags: Cell Biology Source Type: research
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