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Motor unit number index and neurophysiological index as candidate biomarkers of presymptomatic motor neuron loss in amyotrophic lateral sclerosis

Discussion: Mean‐MUNIX and NI were more sensitive than clinical measures at detecting LMN loss in presymptomatic limbs from patients with slowly progressive ALS. Therefore, these electrophysiological biomarkers should be included in early study phases as meaningful outcome measures. Muscle Nerve, 2018
Source: Muscle and Nerve - Category: Internal Medicine Authors: Tags: Clinical Research Article Source Type: research

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In conclusion, we here demonstrated for the first time that human TARDBP mutated MNs can be successfully obtained exploiting the reprogramming and differentiation ability of peripheral blood cells, an easily accessible source from any patient.
Source: Stem Cell Research - Category: Stem Cells Source Type: research
Publication date: 15 June 2018 Source:Life Sciences, Volume 203 Author(s): Qing-Qing Tao, Qiao Wei, Zhi-Ying Wu Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disorder, characterized by the selective degeneration of upper and lower motor neurons. The common clinical symptoms of ALS are caused by the degeneration and dysfunction of motor neurons. With the progression of our understanding of the pathogenesis of the disease, an increasing number of extramotor phenotypes have been linked to ALS. It has long been believed that sensory neurons localized in the dorsal root ganglia are not involved in ALS. In...
Source: Life Sciences - Category: Biology Source Type: research
Publication date: Available online 17 May 2018 Source:Stem Cell Reports Author(s): Evangelos Kiskinis, Joel M. Kralj, Peng Zou, Eli N. Weinstein, Hongkang Zhang, Konstantinos Tsioras, Ole Wiskow, J. Alberto Ortega, Kevin Eggan, Adam E. Cohen Human induced pluripotent stem cell (iPSC)-derived neurons are an attractive substrate for modeling disease, yet the heterogeneity of these cultures presents a challenge for functional characterization by manual patch-clamp electrophysiology. Here, we describe an optimized all-optical electrophysiology, “Optopatch,” pipeline for high-throughput functional characterization ...
Source: Stem Cell Reports - Category: Stem Cells Source Type: research
Abstract The causes of degenerative disease of the upper and lower motor neurons are incompletely understood. In this review the current concepts in the clinical and genetic differential diagnostics of motor neuron diseases are presented. Hereditary spastic paraplegia, primary lateral sclerosis, spinal muscular atrophy and amyotrophic lateral sclerosis are explained, structured according to the affection of the upper and/or lower motor neuron. The substantial variability in the presentation and course of motor neuron diseases as well as the lack of specific laboratory tests hinder an early diagnosis. The precise d...
Source: Der Nervenarzt - Category: Neurology Authors: Tags: Nervenarzt Source Type: research
Conditions:   Amyotrophic Lateral Sclerosis;   ALS;   Lou Gehrig Disease;   Lou Gehrig's Disease;   Lou-Gehrigs Disease;   Motor Neuron Disease, Amyotrophic Lateral Sclerosis Intervention:   Drug: BHV-0223 Sponsors:   Biohaven Pharmaceutical Holding Company Ltd.;   Cognitive Research Corporation (CRC) Recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Abstract The fruit fly Drosophila Melanogaster has been widely used to study neurodegenerative diseases. The conservation of nervous system biology coupled with the rapid life cycle and powerful genetic tools in the fly have enabled the identification of novel therapeutic targets that have been validated in vertebrate model systems and human patients. A recent example is in the study of the devastating motor neuron degenerative disease amyotrophic lateral sclerosis (ALS). Mutations in genes that regulate RNA metabolism are a major cause of inherited ALS, and functional analysis of these genes in the fly nervous sy...
Source: Brain Research - Category: Neurology Authors: Tags: Brain Res Source Type: research
Conclusion: The self-care process in people with MND is not seen in a daily perspective but changes with the evolution of the disease. For the growing patients with MND, changing, accepting and controlling the disease while deciding autonomously are the foundations of the process.
Source: Journal of Neuroscience Nursing - Category: Neuroscience Tags: Article Source Type: research
Publication date: 1 June 2018 Source:Neuroscience, Volume 380 Author(s): Carla Brancia, Barbara Noli, Marina Boido, Roberta Pilleri, Andrea Boi, Roberta Puddu, Francesco Marrosu, Alessandro Vercelli, Paolo Bongioanni, Gian-Luca Ferri, Cristina Cocco While the VGF-derived TLQP peptides have been shown to prevent neuronal apoptosis, and to act on synaptic strengthening, their involvement in Amyotrophic Lateral Sclerosis (ALS) remains unclarified. We studied human ALS patients’ plasma (taken at early to late disease stages) and primary fibroblast cultures (patients vs controls), in parallel with SOD1-G93A transgenic mi...
Source: Neuroscience - Category: Neuroscience Source Type: research
Publication date: 21 June 2018 Source:Neuroscience Letters, Volume 678 Author(s): Stephani A. Davis, Sheed Itaman, Christopher M. Khalid-Janney, Justin A. Sherard, James A. Dowell, Nigel J. Cairns, Michael A. Gitcho Transactive response DNA-binding protein of 43 kDa (TDP-43) functions as a heterogeneous nuclear ribonucleoprotein and is the major pathological protein in frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis/motor neuron disease (ALS/MND). TDP-43 pathology may also be present as a comorbidity in approximately 20–50% of sporadic Alzheimer’s disease cases. In a mouse model of...
Source: Neuroscience Letters - Category: Neuroscience Source Type: research
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