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Amyotrophic Lateral Sclerosis - Europe PMC

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Last Updated: 27 June 2022

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Serum uric acid level predicts the progression of amyotrophic lateral sclerosis following treatment with edaravone.

We investigated whether the treatment effect of edaravone is apparent in patients whose uric acid level increased after the treatment with edaravone. Since edaravone therapy, the Baseline uric acid level and the rate of decline in uric acid were measured. The serum uric acid levels decreased in 26 patients and soared in 12 patients after treatment, and they increased in 12 patients. Patients with uric acid dropped faster in patients whose uric acid was decreased than in patients whose uric acid level increased, but not in patients whose uric acid levels were elevated. Discussion High baseline values and a low rate of decline in uric acid may lead to a slow disease progression in ALS patients treated with edaravone.

Source link: https://europepmc.org/article/MED/35296219


Motor neuron replacement therapy for amyotrophic lateral sclerosis.

Stem cell therapy for amyotrophic lateral sclerosis is a highly cost-effective treatment for both basic and clinical researchers, particularly because transplanted stem cells and stem cell-derived neural progenitor/precursor cells can protect endogenous motor neurons and permanently replace the missing or dying motor neurons. Here, we review the latest advancements in the use of neural stem cells and neural progenitor cells in the treatment of amyotrophic lateral sclerosis. In addition, the significant motor axonal increase after neural progenitor cell transplantation in amyotrophic lateral sclerosis models has shown that motor neuron replacement therapy may be a promising therapeutic therapy for amyotrophic lateral sclerosis models, particularly because a number of stem cell derivatives, including induced pluripotent stem cells, are being considered for clinical trials for various diseases.

Source link: https://europepmc.org/article/MED/35017408


Phosphoinositide-3-kinase regulatory subunit 4 participates in the occurrence and development of amyotrophic lateral sclerosis by regulating autophagy.

Amyotrophic lateral sclerosis can be attributed to multiple proteins' abnormalities. Nevertheless, the role of PIK3R4 in ALS pathogenesis remains uncertain. This research was the first to find that transfection of PC12 cells with small interfering RNA against the PIK3R4 gene dramatically reduced the expression levels of PIK3R4 and the autophagy-related proteins p62 and LC3. In addition, in vivo experiments, the PIK3R4 protein was abundantly expressed in the anterior horn, posterior horn, central canal, and areas surrounding the central canal in adult mice's cervical, thoracic, and lumbar segments. The PIK3R4 protein was predominant in the neural lumbar segments. The onset and onset of ALS disease in Tg1Gur mice were markedly reduced at both the pre-onset and onset stages of ALS disease in Tg1Gur mice relative to control mice, but the progression stage was markedly elevated.

Source link: https://europepmc.org/article/MED/34916448


Protein synthesis modulation as a therapeutic approach for amyotrophic lateral sclerosis and frontotemporal dementia.

Cells must perform life metabolic processes without protein synthesis. Protein synthesis is required for cell function in cells. Some of them represent intricate interactions between endoplasmic reticulum sensors and elongation factors that can rise the expression of chaperone proteins and/or repress translation in order to restore protein homeostasis. The overwhelming majority of cases in amyotrophic lateral sclerosis and frontotemporal dementia show intracellular deposits of ubiquitinated transactive response DNA-binding protein a 43-kDa. TDP-43 is an RNA binding protein that participates in RNA metabolism, among other things. Expression of amyotrophic lateral sclerosis/frontotemporal dementia TDP-43-related mutations in cellular and animal models has been shown to recapitulate key characteristics of the amyotrophic lateral sclerosis/frontotemporal dementia disease spectrum. This article explores the causes of protein imbalance in neurodegenerative disorders and the importance of creating therapeutical compounds aimed at restoring proteostasis.

Source link: https://europepmc.org/article/MED/34916412


MicroRNA biomarkers in frontotemporal dementia and to distinguish from Alzheimer's disease and amyotrophic lateral sclerosis.

Frontotemporal lobar degeneration is a group of chronic brain disorders that are mainly related to atrophy of the prefrontal and anterior temporal lobes. Frontotemporal lobar degeneration is thought to be comparable to frontotemporal dementia. For developing treatment plans and interventions for these patients, the early diagnosis of frontotemporal dementia is vital. Healthy controls, Alzheimer's disease, and amyotrophic lateral sclerosis can be distinguished from specific miRNAs isolated or in combination, or as miRNA pairs in blood plasma, serum, or cerebrospinal fluid enabled frontotemporal dementia. Frontotemporal lobar degeneration and frontotemporal dementia, respectively, were distinguished by downregulation of miR-132-3p in frontal and temporal cortical tissue, according to healthy controls. MiR-223-3p, miR-15a-5p, miR-22-3p, cerebrospinal fluid, and miR-124 in cerebrospinal fluid are all potential promising miRNA biofluid biomarker candidates for behavioral-variant frontotemporal dementia. No miRNAs were found to distinguish between behavioral-variant frontotemporal dementia and primary progressive aphasia subtypes.

Source link: https://europepmc.org/article/MED/34916411


Alteration of interoceptive sensitivity: expanding the spectrum of behavioural disorders in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis is a neurodegenerative disorder with progressive loss of upper and lower motor neurons. Objects: Objectives The heartbeat perception task is used to assess IS in ALS patients and is based on a well-established task. Methods Fifty-five patients and 41 caregivers underwent the heartbeat perception test as well as an extensive investigation of motor, cognitive, body consciousness, affective, and emotion domains. AS patients had lower IS than CG compared to CG. Significant correlations were found between IS and self-reported measures of alexia and interoceptive awareness, which showed strong correlations. No significant differences were found on questionnaires for depression and anxiety among patients with ALS and their caregivers. Despite cognitive and motor impairment, ALS patients' increased interoceptive sensitivity, decreased empathy, and less focused on pain. Alteration of interoception can be a specific behavioral characteristic present in ALS patients.

Source link: https://europepmc.org/article/MED/35751711


Repeated neurofilament light chain measurements did not capture Riluzole therapeutic effect in amyotrophic lateral sclerosis patients.

The comparison was made between patients with Riluzole at inclusion in the study and those who were treated with Riluzole following inclusion. The results showed that sNfL levels in ALS patients were higher than in controls at the two time points. Both sNfL1 and snfl2 were higher in females in ALS patients. SnfL2 levels were higher at sNfL2 than at sNfL1 in the whole ALS group. Conclusions This study found that sNfL levels in ALS patients increased with time, but there was no difference between subjects treated by Riluzole and those treated after sNfL1. To better determine the true ability of sNfL measurement as a way to monitor treatment response in ALS, further research with larger population samples and different sampling intervals are required.

Source link: https://europepmc.org/article/MED/35751632


Retromer dysfunction in amyotrophic lateral sclerosis.

Here, we show a decrease in the retromer proteins-vacuolar protein sorting 35, VPS26A, and VPS29-in patients with amyotrophic lateral sclerosis and in the ALS model developed by transgenic mice expressing the mutant superoxide dismutase-1 G93A. In Tg SOD1 G93A mice, a viral vector expressing VPS35 promotes the retromer deficit, which leads to the paralytic phenotype. Conversely, lowering Vps35 levels in Tg SOD1 G93A mice improves the disease phenotype, although decreasing Vps35 levels increases the disease phenotype.

Source link: https://europepmc.org/article/MED/35749364


Integrated multi-omic data analysis and validation with yeast model show oxidative phosphorylation modulates protein aggregation in amyotrophic lateral sclerosis.

Amyotrophic Lateral Sclerosis is a progressive, incurable amyloid aggregating neurodegenerative disorder affecting the motor neurons. Deregulation of Non-alcoholic fatty acid liver disease and oxidative phosphorylation was found in our analysis of ALS patient data. SOD1, FUS, and TDP-43 mice studies, as well as TDP-43, showed deregulation of oxidative phosphorylation and ribosome-associated pathways. In addition, a network analysis of mitochondrial electron transport chain revealed enrichment of proteins and protein-drug interaction networks. Complex III and IV of mitochondrial Complex III and IV. Metformin and malonate did not have any effect in reducing the amyloid generation, according to amyloid researchers, although antibiotic and azide reduced amyloidogenesis.

Source link: https://europepmc.org/article/MED/35749136


A pathologically confirmed case of combined amyotrophic lateral sclerosis with C9orf72 mutation and multiple system atrophy.

In C9orf72 cases, a substantial proportion of cases of amyotrophic lateral sclerosis and frontotemporal lobar degeneration is due to repeated expansions. However, pathological evidence of a parkinsonian syndrome associated with a C9orf72 repeat expansion has only been found very rarely. There have been no reported pathologically confirmed cases of ALS with C9orf72 mutation and multiple system atrophy to date, as far as we know. We discuss a man who appeared with extrapyramidal characteristics, including cogwheel rigidity, and, as a result, was clinically diagnosed with Parkinson's disease or parkinsonian syndrome. The brain and spinal cord's macroscopic and microscopic examination revealed ALS pathologie with neuronal loss, particularly of the anterior horns of the cord and the motor cortex. The inclusions were identified with a number of neuronal cytoplasmic inclusions immunoreactive for phosphorylated transactivation response DNA-binding protein of 43 Da.

Source link: https://europepmc.org/article/MED/35746899

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions