Supply bottleneck impairs nerve function

(University of W ü rzburg) Impaired transport processes in neurons contribute to diseases such as amyotrophic lateral sclerosis (AML). W ü rzburg scientists have now identified key actors in these processes.
Source: EurekAlert! - Medicine and Health - Category: International Medicine & Public Health Source Type: news

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Astrocytes are involved in non-cell-autonomous pathogenic cascades in amyotrophic lateral sclerosis (ALS); however, their role is still debated. We show that astrocytic NF-B activation drives microglial proliferation and leukocyte infiltration in the SOD1 (G93A) ALS model. This response prolongs the presymptomatic phase, delaying muscle denervation and decreasing disease burden, but turns detrimental in the symptomatic phase, accelerating disease progression. The transition corresponds to a shift in the microglial phenotype showing two effects that can be dissociated by temporally controlling NF-B activation. While NF-B ac...
Source: EMBO Journal - Category: Molecular Biology Authors: Tags: Molecular Biology of Disease, Neuroscience Articles Source Type: research
The role of astrocytes and microglia in neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) remains incompletely understood. In this issue of The EMBO Journal, Alami et al (2018) employ a sophisticated genetic system that allows precise temporal control of NF-B activation in astrocytes to demonstrate that the timing of astrocyte activation is a key determinant of disease progression. Their results suggest that astrocyte activation drives microglia proliferation and that this can amplify not only the protective microglial effects in the presymptomatic phase of ALS, but also the detrimental microglial...
Source: EMBO Journal - Category: Molecular Biology Authors: Tags: Molecular Biology of Disease, Neuroscience News [amp ] Views Source Type: research
Publication date: Available online 14 August 2018Source: Neurochemistry InternationalAuthor(s): Lindsay Joy Spielman, Deanna Lynn Gibson, Andis KlegerisAbstractThe number of bacterial cells living within the human body is approximately equal to, or greater than, the total number of human cells. This dynamic population of microorganisms, termed the human microbiota, resides mainly within the gastrointestinal tract. It is widely accepted that highly diverse and stable microbiota promote overall human health. Colonization of the gut with maladaptive and pathogenic microbiota, a state also known as dysbiosis, is associated wit...
Source: Neurochemistry International - Category: Neuroscience Source Type: research
Bruna De Felice, Francesco Manfellotto, Giuseppe Fiorentino, Anna Annunziata, Elio Biffali, Raimondo Pannone, Antonio Federico
Source: Frontiers in Genetics - Category: Genetics & Stem Cells Source Type: research
Otorhinolaryngologic examinations at an early stage, particularly those conducted by vocal specialists, can make potentially important contributions to the diagnosis of bulbar-onset ALS patients.
Source: Auris, Nasus, Larynx - Category: ENT & OMF Authors: Source Type: research
To the Editor We read with great interest the article by Rosenbohm and colleagues. They conducted a case-control study and reported that retinol-binding protein 4 had an inverse association with both risk for amyotrophic lateral sclerosis (ALS) and disease prognosis. Although we agree with the relevance of the reported results and their interpretation, we would like to comment on 2 issues that might also correlate with these findings.
Source: JAMA Neurology - Category: Neurology Source Type: research
In Reply We appreciate the comments of Riancho et al on our article investigating the association of retinol-binding protein 4 (RBP4) concentrations with the amyotrophic lateral sclerosis (ALS) risk and prognosis in the ALS registry Swabia. In their letter, they pointed out important clinical aspects and suggested that the causes of the disease could vary depending on the clinical characteristics of ALS and should be considered.
Source: JAMA Neurology - Category: Neurology Source Type: research
Abstract Transactive response DNA/RNA-binding protein 43-kDa (TDP-43) C-terminal fragments, such as a 25-kDa fragment (TDP-25), have been identified as a ubiquitinated and phosphorylated components of inclusion bodies (IBs) in motor neurons from amyotrophic lateral sclerosis patients. Cells contain proteins that function as molecular chaperones and prevent aggregate formation of misfolded and aggregation-prone proteins. Recently, we reported that heat shock protein (HSP)70, an abundant molecular chaperone, binds to TDP-25 in an ATP-dependent manner; however, whether HSP70 can prevent the formation of TDP-25-relate...
Source: Cell Stress and Chaperones - Category: Cytology Authors: Tags: Cell Stress Chaperones Source Type: research
Amyotrophic lateral sclerosis (ALS), the most common degenerative disease affecting motor neurons, lacks an effective disease-modifying disease treatment [1 –3]. Recently, the Src/c-Abl pathway has been postulated as a potential therapeutic target in ALS. [4]. Imamura et al. developed a phenotypic screen to repurpose existing drugs for ALS patients, which resulted in the identification of 27 potential hits. Intriguingly, more than half of the hits tar geted the Src/c-Abl signaling pathway [4]. Complementary studies demonstrated that Src/c-Abl inhibitors increased the survival of induced pluripotent stem cells (iPSC)-...
Source: Journal of the Neurological Sciences - Category: Neurology Authors: Tags: Letter to the Editor Source Type: research
Condition:   Amyotrophic Lateral Sclerosis Interventions:   Drug: BIIB078;   Drug: Placebo Sponsor:   Biogen Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
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