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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Conclusion: Overall, and even though we must account for the limitations of the indirect methods and models used for prevalence estimation, we probably have a very high ALS/MND prevalence in Portugal. It would be important to create registries, particularly in rare diseases, for better organization and distribution of healthcare services and resources, particularly at the level of ventilatory support.Neuroepidemiology
Source: Neuroepidemiology - Category: Epidemiology Source Type: research
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Source: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration - Category: Neurology Authors: Source Type: research
Conclusion: This study provides evidence that not all ALS patients show contiguous clinical or electrophysiological spread patterns. The electrophysiological spread pattern can affect the functional staging in ALS patients. Keywords:Amyotrophic lateral sclerosis, prion-like mechanism, electrophysiology, spread pattern
Source: Frontiers in Neurology - Category: Neurology Source Type: research
Amyotrophic lateral sclerosis (ALS) is the most common motor neuron degenerative disease in adults and has also been proven to be a type of conformational disease associated with protein misfolding and dysfunction. To date, more than 150 distinct genes have been found to be associated with ALS, among which Superoxide Dismutase 1 (SOD1) is the first and the most extensively studied gene. It has been well established that SOD1 mutants-mediated toxicity is caused by a gain-of-function rather than the loss of the detoxifying activity of SOD1. Compared with the clear autosomal dominant inheritance of SOD1 mutants in ALS, the po...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
Abstract OBJECTIVE: The aims of this study were to identify the unmet care needs and to examine the mediating effect of unmet supportive care needs in the relationship between functional status and quality of life (QOL) in Korean patients with amyotrophic lateral sclerosis (ALS). METHOD: This was a cross-sectional study conducted among 186 patients with ALS recruited from a tertiary hospital in Seoul, South Korea. ALS patients' functional status, unmet supportive care needs, and QOL were assessed by Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised, the Amyotrophic Lateral Sclerosis Supportive Car...
Source: Palliative and Supportive Care - Category: Palliative Care Authors: Tags: Palliat Support Care Source Type: research
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
In conclusion, our data show that continuous G-CSF treatment fails to increase circulating CD34+ HSPC in ALS patients. Cytokine profiles revealed G-CSF-mediated immunomodulatory and proteolytic effects. Interestingly, despite intense G-CSF stimulation, telomere length was not significantly shortened. PMID: 31100684 [PubMed - as supplied by publisher]
Source: Cytokine - Category: Molecular Biology Authors: Tags: Cytokine Source Type: research
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