Trying to understand cells' interior design

(Institute for Basic Science) IBS Scientists have explained how liquid-like droplets made of proteins and DNA form in vitro. Currently, there is a huge interest in understanding the molecular mechanisms behind the creation of such droplets, as it is linked to some human diseases, such as amyotrophic lateral sclerosis (ALS). The results showed how much the sequence of DNA matters in the formation of such droplets.
Source: EurekAlert! - Biology - Category: Biology Source Type: news

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Conclusion: Our results do not support the causal role of genetically increased or decreased BMI on the risk of ALS.
Source: Frontiers in Neurology - Category: Neurology Source Type: research
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
Amyotrophic lateral sclerosis (ALS) is a debilitating disease with few treatment options. Progress towards new therapies requires validated disease biomarkers, but there is no consensus on which fluid-based me...
Source: Journal of Translational Medicine - Category: Research Authors: Tags: Research 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
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