Spinal excitability changes do not influence the mechanisms of split ‐hand syndrome in amyotrophic lateral sclerosis

Muscle&Nerve, EarlyView.
Source: Muscle and Nerve - Category: Internal Medicine Authors: Source Type: research

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by Peng Wang, Jianwen Deng, Jie Dong, Jianghong Liu, Eileen H. Bigio, Marsel Mesulam, Tao Wang, Lei Sun, Li Wang, Alan Yueh-Luen Lee, Warren A. McGee, Xiaoping Chen, Kazuo Fushimi, Li Zhu, Jane Y. Wu Mutations in or dys-regulation of the TDP-43 gene have been associated with TDP-43 proteinopathy, a spectrum of neurodegenerative diseases including Frontotemporal Lobar Degeneration (FTLD) and Amyotrophic Lateral Sclerosis (ALS). The underlying molecular and cellular defects, however, remain uncl ear. Here, we report a systematic study combining analyses of patient brain samples with cellular and animal models for TDP-43 pro...
Source: PLoS Genetics - Category: Genetics & Stem Cells Authors: Source Type: research
Many neurodegenerative disorders, including Parkinson ’s, Alzheimer’s, and amyotrophic lateral sclerosis, are well known to involve the accumulation of disease-specific proteins. Less well known are the accumul...
Source: Molecular Neurodegeneration - Category: Neurology Authors: Tags: Review Source Type: research
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Source: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration - Category: Neurology Authors: Source Type: research
AbstractObjectiveSeveral independent prognostic factors, such as age of onset, type of onset, body mass index (BMI), and progression rate have been identified for amyotrophic lateral sclerosis (ALS) in Caucasians. The aim of this study was to identify such factors in Chinese patients and to compare their impact with German patients.MethodsComparison of prognostic factors was based on two hospital-based registries. The registry of the German Network for Motor Neuron Diseases contains 3100 patients with ALS. The Chinese registry comprises 2101 patients who were collected between 2003 and 2015 in the metropolitan area of Beij...
Source: Journal of Neurology - Category: Neurology Source Type: research
Amyotrophic lateral sclerosis (ALS), also known as motor neurone disease, is an incurable neurodegenerative condition, characterized by the loss of upper and lower motor neurons. It affects 1-1.8/100,000 individuals worldwide, and the number of cases is projected to increase as the population ages. Thus, there is an urgent need to identify both therapeutic targets and disease-specific biomarkers - biomarkers that would be useful to diagnose and stratify patients into different sub-groups for therapeutic strategies, as well as biomarkers to follow the efficacy of any treatment tested during clinical trials. There is a lack ...
Source: Frontiers in Neurology - Category: Neurology 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
In this study, a significant (30%) increase in maximum lifespan of mice was found after nonablative transplantation of 100 million nucleated bone marrow (BM) cells from young donors, initiated at the age that is equivalent to 75 years for humans. Moreover, rejuvenation was accompanied by a high degree of BM chimerism for the nonablative approach. Six months after the transplantation, 28% of recipients' BM cells were of donor origin. The relatively high chimerism efficiency that we found is most likely due to the advanced age of our recipients having a depleted BM pool. In addition to the higher incorporation rates, ...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
Publication date: Available online 11 May 2019Source: Seminars in Cell &Developmental BiologyAuthor(s): Charannya Sozheesvari Subhramanyam, Cheng Wang, Qidong Hu, S Thameem DheenAbstractMicroglia, being the resident immune cells of the central nervous system, play an important role in maintaining tissue homeostasis and contributes towards brain development under normal conditions. However, when there is a neuronal injury or other insult, depending on the type and magnitude of stimuli, microglia will be activated to secrete either proinflammatory factors that enhance cytotoxicity or anti-inflammatory neuroprotective fac...
Source: Seminars in Cell and Developmental Biology - Category: Cytology Source Type: research
Publication date: Available online 9 May 2019Source: Stem Cell ReportsAuthor(s): Ilary Allodi, Jik Nijssen, Julio Aguila Benitez, Christoph Schweingruber, Andrea Fuchs, Gillian Bonvicini, Ming Cao, Ole Kiehn, Eva HedlundSummaryOculomotor neurons, which regulate eye movement, are resilient to degeneration in the lethal motor neuron disease amyotrophic lateral sclerosis (ALS). It would be highly advantageous if motor neuron resilience could be modeled in vitro. Toward this goal, we generated a high proportion of oculomotor neurons from mouse embryonic stem cells through temporal overexpression of PHOX2A in neuronal prog...
Source: Stem Cell Reports - Category: Stem Cells Source Type: research
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