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Wednesday, February 07, 2018 -- Scientists have for the first time discovered a mechanism that limits the number of "cellular janitors" in the nervous system, leading to increased risk for two neurodegenerative diseases: amyotrophic lateral sclerosis and frontotemporal dementia, according to a Keck School of Medicine of USC study published today in Nature Medicine.
Wednesday, February 07, 2018 -- Emerging NMJ in vitro models aim to help scientists discover drugs that help the muscles and motor neurons stay connected in ALS.
Tuesday, February 06, 2018 -- Biogen expands its ALS portfolio by licensing Karyopharm's KPT-350 which targets nuclear export.
Monday, February 05, 2018 -- Mallinckrodt plc confirmed enrollment of the first patient in the company's Phase 4, multi-center, randomized, double-blind, placebo-controlled pilot study to further assess the efficacy of H.P. Read more..
Saturday, February 03, 2018 -- The C9orf72 DRP polyGA soaks up proteasomes according to cryoET analysis, potentially explaining why misfolded proteins may accumulate in neurons in C9orf72 ALS.
Friday, February 02, 2018 -- Low muscle strength during the later teen years has been identified as a risk factor for much later onset of the neurological disease known as ALS, or amyotrophic lateral sclerosis. A study at Sahlgrenska Academy published in the Journal of Neurology also links low blood counts at a young age to ALS.
Wednesday, January 24, 2018 -- Restoring mitochondrial dynamics in motor neurons may help protect them against ALS according to a new study.
Tuesday, January 23, 2018 -- Kif5A variants are linked to ALS, suggesting that a disruption in axonal transport leads to at least one form of the disease.
Friday, January 19, 2018 -- Scientists say they have described for the first time atom-by-atom changes in a protein family linked to amyotrophic lateral sclerosis (ALS). Their study (“ Mechanistic View of hnRNPA2 Low-Complexity Domain Structure, Interactions, and Phase Separation Altered by Mutation and Arginine Methylation ”) is published in Molecular Cell. “hnRNPA2, a component of RNA-processing membraneless organelles, forms inclusions when mutated in a syndrome characterized by the degeneration of neurons (bearing features of amyotrophic lateral sclerosis [ALS] and frontotemporal dementia), muscle, and bone. Here we provide a unified structural view of hnRNPA2 self-assembly, aggregation, and interaction and the distinct effects of small chemical changes—disease mutations and arginine methylation—on these assemblies,” write the investigators. “The hnRNPA2 low-complexity (LC) domain is compact and intrinsically disordered
Monday, January 15, 2018 -- Researchers at Western University believe they have found a common link between the degenerative brain condition CTE, and a variant of ALS, or Lou Gehrig’s disease.
Thursday, January 11, 2018 -- BACKGROUND AND PURPOSE: There is an emerging need for biomarkers to better categorize clinical phenotypes and predict progression in amyotrophic lateral sclerosis. This study aimed to quantify cervical spinal gray matter atrophy in amyotrophic lateral sclerosis and investigate its association with clinical disability at baseline and after 1 year. MATERIALS AND METHODS: Twenty-nine patients with amyotrophic lateral sclerosis and 22 healthy controls were scanned with 3T MR imaging. Standard functional scale was recorded at the time of MR imaging and after 1 year. MR imaging data were processed automatically to measure the spinal cord, gray matter, and white matter cross-sectional areas. A statistical analysis assessed the difference in cross-sectional areas between patients with amyotrophic lateral sclerosis and controls, correlations
Thursday, January 11, 2018 -- BACKGROUND AND PURPOSE: Amyotrophic lateral sclerosis is a neurodegenerative disease involving the upper and lower motor neurons. In amyotrophic lateral sclerosis, pathologic changes in the primary motor cortex include Betz cell depletion and the presence of reactive iron-loaded microglia, detectable on 7T MR images as atrophy and T2*-hypointensity. Our purposes were the following: 1) to investigate the signal hypointensity-to-thickness ratio of the primary motor cortex as a radiologic marker of upper motor neuron involvement in amyotrophic lateral sclerosis with a semiautomated method at 3T, 2) to compare 3T and 7T results, and 3) to evaluate whether semiautomated measurement outperforms visual image assessment. MATERIALS AND METHODS: We investigated 27 patients and 13 healthy subjects at 3T, and 19 patients and
Wednesday, January 10, 2018 -- Cognitive challenges may occur in ALS due to the loss of synapses in the brain and therefore, may not occur indirectly due to the loss of neurons.
Tuesday, January 09, 2018 -- Neurodegeneration in ALS/FTD may be caused by TDP-43 aggregation in the cytoplasm, causing cellular traffic jams. The trial drug can sequester TDP-43.
Friday, January 05, 2018 -- Pfizer and Sangamo plan to develop a potential gene therapy for C9orf72 ALS that aims to protect motor neurons by reducing levels of repeat-rich RNAs.
Thursday, January 04, 2018 -- ALS is a debilitating and ultimately deadly neural disease that has few treatments and no cure.
Thursday, January 04, 2018 -- Patients older than 65 were more likely to have steeper declines in slow vital capacity.
Thursday, January 04, 2018 -- Pfizer and Sangamo Therapeutics are linking arms to develop a potential gene therapy to treat amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD).
Wednesday, January 03, 2018 -- (Reuters) - Sangamo Therapeutics Inc and Pfizer Inc said on Wednesday they would work together to develop a gene therapy to treat ALS, a disease that affects nerve cells in the brain and the spinal cord.
Tuesday, December 26, 2017 -- Scientists have revealed more details of the molecular mechanism behind neuronal cell death in amyotrophic lateral sclerosis (ALS), a step forward to find ways to control progression of the disease.