Results 91 to 100 of about 235,642 (386)

The human gut microbiota in people with amyotrophic lateral sclerosis

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2020
Objective To characterize the gut microbiota in people with amyotrophic lateral sclerosis (ALS) relative to controls and to test the hypothesis that butyrate-producing bacteria are less abundant in the gastrointestinal tracts of people with ALS (PALS ...
K. Nicholson, K. Bjørnevik, G. Abu-Ali, James Chan, Marianna Cortese, Brixhilda Dedi, M. Jeon, R. Xavier, C. Huttenhower, A. Ascherio, J. Berry   +10 more
semanticscholar   +1 more source

Pain in amyotrophic lateral sclerosis [PDF]

The Lancet Neurology, 2017
Pain is a largely neglected symptom in patients with amyotrophic lateral sclerosis (ALS) although it is reported by most of these patients. It occurs at all stages of the disease and can be an onset symptom preceding motor dysfunction. Pain is correlated with a deterioration in patients' quality of life and increased prevalence of depression.
CHIO', Adriano, Mora, Gabriele, LAURIA, GIUSEPPE   +2 more
openaire   +2 more sources

The Diverse Neuromuscular Spectrum of VPS13A Disease

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective VPS13A disease (chorea‐acanthocytosis) is a rare neurodegenerative disorder caused by biallelic variants in VPS13A, typically presenting with hyperkinetic movement disorders, while neuromuscular signs are often mild. The aim of the project was to investigate the frequency and severity of neuromuscular impairment in VPS13A disease ...
Anne Buchberger, Evamaria Riedel, Marie Hackenberg, Alexander Mensch, Stefanie Beck‐Woedl, Joohyun Park, Tobias B. Haack, Bernhard Haslinger, Jan Kirschke, Holger Prokisch, Andreas Hermann, Christian Mawrin, Adrian Danek, Benedikt Schoser, Kevin Peikert, Marcus Deschauer, Isabell Cordts   +16 more
wiley   +1 more source

Circulating proteomic biomarkers for diagnosing sporadic amyotrophic lateral sclerosis: a cross-sectional study

Neural Regeneration Research
Biomarkers are required for the early detection, prognosis prediction, and monitoring of amyotrophic lateral sclerosis, a progressive disease. Proteomics is an unbiased and quantitative method that can be used to detect neurochemical signatures to aid in
Lu He, Qinming Zhou, Chaoyang Xiu, Yaping Shao, Dingding Shen, Huanyu Meng, Weidong Le, Sheng Chen   +7 more
doaj   +1 more source

Viral delivery of antioxidant genes as a therapeutic strategy in experimental models of amyotrophic lateral sclerosis. [PDF]

, 2013
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder with no effective treatment to date. Despite its multi-factorial aetiology, oxidative stress is hypothesized to be one of the key pathogenic mechanisms.
Azzouz, M, Higginbottom, A, Nanou, A, Ning, K, Shaw, P, Valori, CF, Wyles, M   +6 more
core   +1 more source

Exosome Proteomics of SOD1D90A Mutation Suggest Early Disease Mechanisms, and FN1 as a Biomarker

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Amyotrophic lateral sclerosis (ALS) is a neuromuscular disease. Super oxide dismutase 1 (SOD1) gene mutations cause ALS, and the D90A mutation is associated with primarily upper motor neuron (UMN) loss. Objective Our goal is to reveal the early cellular events in ALS pathology and identify potential pharmacokinetic biomarkers, using well ...
Mukesh Gautam, Ali Laith, Aslihan Gunel, Melda Yilmaz, Nazli Basak, Halil Idrisoglu, P. Hande Ozdinler   +6 more
wiley   +1 more source

C9orf72 hexanucleotide repeat allele tagging SNPs: Associations with ALS risk and longevity

Frontiers in Genetics, 2023
C9orf72 hexanucleotide repeat expansion is a common cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). The C9orf72 locus may harbor residual risk outside the hexanucleotide repeat expansion, but the evidence is conflicting ...
Karri Kaivola, Karri Kaivola, Matti Pirinen, Matti Pirinen, Matti Pirinen, Hannu Laaksovirta, Lilja Jansson, Lilja Jansson, Osma Rautila, Osma Rautila, Jyrki Launes, Laura Hokkanen, Jari Lahti, Johan G. Eriksson, Johan G. Eriksson, Johan G. Eriksson, Johan G. Eriksson, Timo E. Strandberg, Timo E. Strandberg, FinnGen, Pentti J. Tienari, Pentti J. Tienari   +21 more
doaj   +1 more source

Neuron-Specific HuR-Deficient Mice Spontaneously Develop Motor Neuron Disease [PDF]

, 2018
Human Ag R (HuR) is an RNA binding protein in the ELAVL protein family. To study the neuron-specific function of HuR, we generated inducible, neuron-specific HuR-deficient mice of both sexes.
Bai, Ying, Chen, Xing, Hamilton, Thomas A., Herjan, Tomasz, Kokiko-Cochran, Olga N., Lamb, Bruce, Lee, Yu-Shang, Li, Xiao, Lin, Ching-Yi, Sun, Kevin, Zhou, Gao   +10 more
core   +1 more source

Growth Differentiation Factor 15 Elevation in the Central Nervous System Is Associated With Failure to Thrive in Alexander Disease

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Alexander disease (AxD) is a severe neurodegenerative disorder caused by gain‐of‐function mutations in the gene for GFAP, which lead to protein aggregation and a primary astrocytopathy. Symptoms vary, but failure to thrive (FTT) and frequent emesis are common and cause significant morbidity. Here we investigate GDF15, a member of the
Tracy L. Hagemann, Michelle M. Sonsalla, Cora Luzinski, Fernando Zacahua, David A. Harris, Dudley W. Lamming, Albee Messing   +6 more
wiley   +1 more source

ALS mutant FUS proteins are recruited into stress granules in induced pluripotent stem cells- derived motoneurons [PDF]

, 2015
Patient-derived induced Pluripotent Stem Cells (iPSCs) provide an opportunity to study human diseases mainly in those cases where no suitable model systems are available.
Bozzoni, Irene, Caliendo, Virginia, Calvo, Andrea, Chiò, Adriano, DE SANTIS, Riccardo, Laneve, Pietro, Lenzi, Jessica, Morlando, Mariangela, Rosa, Alessandro, Turris, Valeria de   +9 more
core   +4 more sources