Advanced searches left 3/3

Amyotrophic Lateral Sclerosis - DOAJ

Summarized by Plex Scholar
Last Updated: 27 June 2022

* If you want to update the article please login/register

Early presentation of lower urinary tract and bowel dysfunction in sporadic amyotrophic lateral sclerosis: A case report

An autopsy report of sporadic amyotrophic lateral sclerosis with lower urinary tract and bowel dysfunction has been reported. In the early stages of the disease, the dysfunction coincided with motor neuron problems. Neuronal degeneration and gliosis were found in the thoracic and sacral nucleus, as well as in Onuf's nucleus, and in Onuf's nucleus, although gliosis was present in the periaqueductal gray and striatum, according to neuropathological results, although gliosis was not present in the thoracic and sacral nucleus, not shown in the striatum and chronal decay and gliogratis in the glio glios and gliotus and gliolog glio chon chond's nucleus nucleus, striatum, striatus. Positive inclusions were found in the peripheral nerves of the thoracic sympathetic ganglia, as well as the IML of the thoracic spinal cord. Although it is impossible to determine the precise neuropathological signs relating to LUT and bowel dysfunction, physicians must understand that neurogenic bladder and bowel dysfunction can occur in patients with ALS.

Source link: https://doi.org/10.1016/j.ensci.2022.100413


Lipid Metabolism Is Dysregulated in the Motor Cortex White Matter in Amyotrophic Lateral Sclerosis

In amyotrophic lateral sclerosis, metabolism is profoundly impaired, but the white matter, where the myelinated axons of motor neurons are located, remains uncharacterized. Whilst myelin protein composition was similar in ALS and control tissue, both the lipid levels and enzyme expression of their corresponding enzymes were dysregulated, highlighting elevated lipid metabolism in the white matter as well as a potential change in myelin composition. Altered myelin composition could cause motor neuron dysfunction, and this shows how oligodendrocytes can play a vital role in ALS pathogenesis.

Source link: https://doi.org/10.3390/metabo12060554


Metabolic Profile and Pathological Alterations in the Muscle of Patients with Early-Stage Amyotrophic Lateral Sclerosis

We established the metabolome of muscle and serum of ALS patients and compared these results with the clinical condition and pathological changes observed in the muscle. SOD3 and GLRX2 upregulation in ALS muscle was demonstrated by Transcriptomics analysis of key antioxidant enzymes. In ALS, mitochondrial enzyme activity in muscle revealed higher complicated II/CS and lower LDH levels in ALS than in controls. For the first time, our study revealed a global defect in early-stage ALS patients' muscles.

Source link: https://doi.org/10.3390/biomedicines10061307


Transplantation of Human Glial Progenitors to Immunodeficient Neonatal Mice with Amyotrophic Lateral Sclerosis (SOD1/rag2)

Amyotrophic lateral sclerosis is a progressive, fatal disease with no effective treatment. ALS' neurodegenerative physiology was an attractive target for stem cell-based regenerative approaches. In both preclinical and clinical settings, various types of stem cell transplants have been transplanted, but no definitive results have been reported. Human glial isolated precursors transplanted intraventricularly to neonatal, immunodeficient mice extended the lifespan of dysmyelinated mice by a year. hGRP injections into the mouse model of ALS also provided benefits. We have recently introduced an immunodeficient mouse model of ALS, and in this research, we also tested the approach that had been used in dysmyelinated mice of intraventricular transplantation of hGRPs to immunodeficient mice. Grafting into neonatal immunodeficient recipients did not prevent ALS-mediated cell death, which may have explained the lack of promising therapeutic results. Therefore, we encourage stem cell and ALS groups to research and implement cell tracking tools to help diagnose cell fates in the clinic.

Source link: https://doi.org/10.3390/antiox11061050


Causal Inference of Genetic Variants and Genes in Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis is a fatal progressive multisystem disease with limited therapeutic options. Despite numerous ALS susceptibility loci, the precise identities of causal variants, genes, cell types, and tissues, as well as their physiological roles in ALS formation remain largely unknown, despite genome-wide association studies. Here, we published a comprehensive post-GWAS review of the new large ALS GWAS, which included functional mapping and annotation, transcriptome-wide association study, colocalization, and summary data-based Mendelian randomization analyses in large multi-omics datasets. Functional cell types of ALS and confirmed cerebellum and cerebellar hemisphere as functional tissues of ALS, according to gene property analysis. We also suggested that rs2453555 at 9p21. 2 and SCFD1 in skeletal muscle develops ALS by tightly limiting the expression of C9orf72 in pituitary and SCFD1, respectively.

Source link: https://doi.org/10.3389/fgene.2022.917142


Analysis on Acupoint Selection and Combination for Amyotrophic Lateral Sclerosis Treated with Acupuncture Based on Data Mining

By using data mining techniques, the aim of the study was to investigate the regularity of acupoints used in the diagnosis of amyotrophic lateral sclerosis. The scientific literature proving acupuncture in the treatment of ALS was eligible for inclusion. For statistical analysis, modular data mining techniques, such as acupoint frequency, intricate network analysis, association rule construction, and cluster analysis were used. Forty-two literature reviews on 141 acupoints were included, with 626 times the average application frequency. Hand Yangming's most visible meridian was the large intestine meridian. Hen-Quchi, Quchi-Zusanli, Zusanli-Sanyinjiao, Hesanli-Sanyinjiao, Hesanli, Hesanli-Sanyinjiao, Hesanyi, Quchi-Quchi, Quchi-Zusanli-Zusanli-Sanyi-Sanyi-Sanyi-Sanyi-Sanyi, Hesanyi-Sanyiao, Hesanli-Sanyi, Hesanyi, Hesanli, Hesanyi, Hesanli, Heman-Sanyi-Sanyiao, and Quchi-Sanyi Sanyi, He-Sanyi, Hem According to the cluster report, there were 7 acupoint groups. Hegu, Zusanli, Quchi, and Jiaji can be used as the key prescriptions in treating ALS. In acupuncture therapy, the combination of Quchi, Hegu, Zusanli, and Sanyinjiao should be chosen with priority.

Source link: https://doi.org/10.1155/2022/6541600


Functional alterations in large-scale resting-state networks of amyotrophic lateral sclerosis: A multi-site study across Canada and the United States

Amyotrophic lateral sclerosis is a multisystem neurodegenerative disorder characterized by progressive degeneration of upper motor neurons and lower motor neurons, as well as frontotemporal regions, which lead to impaired bulbar, leg, and cognitive function. Cortical and subcortical brain involvement in ALS pathophysiology has been documented in magnetic resonance imaging studies. Through the Canadian ALS Neuroimaging Consortium, a total of 120 subjects 120 ALS patients were recruited across North America; 115 healthy controls were recruited across North America. In addition, ALS patients had elevated inter-network RSNs between the orbital and basal ganglia RSNs, which negatively related to cognitive impairment. In summary, there is an increase in intra- and inter-network functional connectivity of RSNs underpinning both motor and cognitive impairment.

Source link: https://doaj.org/article/70ccadd2366f47ac9f86a27e1714a364


Edaravone efficacy in amyotrophic lateral sclerosis with reduced forced vital capacity: Post-hoc analysis of Study 19 (MCI186-19) [clinical trial NCT01492686]

In phase 3 of MCI186-19, Edaravone slowed the rate of functional decline in patients with amyotrophic lateral sclerosis. Objective In ALS patients with FVC > 80%, the question of equitability of edaravone in ALS patients with FVC 80%p. Methods Affective Study 19 comparing edaravone effectiveness at week 48 in subjects with FVC24 u226580%p> vs 80%p. Through week 48, participants in both the FVC24 u226580%p and the FVC24 80%p subgroups experienced a decrease in ALS Functional Rating Scale-Revised score loss vs placebo subjects. For the FVC24 80%p subgroup, the changes in ALSFRS-R scores from baseline to week 48 were u22127. 63, a difference of 2. 05 percent. Conclusion Conclusions ALS patients in the Study 19 placebo group had a decrease in disease progression even after edaravone was introduced with a FVC of 80%p prior to starting edaravone.

Source link: https://doaj.org/article/b506e89852f54f6b834ad7991d39383e


Clinical characteristics and prognosis of amyotrophic lateral sclerosis with autoimmune diseases.

Introduction The prevalence of autoimmune disorders in amyotrophic lateral sclerosis patients has been widely reported, but little is known about the underlying clinical phenotype. This research seeks to investigate the clinical characteristics and prognosis of ALS patients with AID. At registration, ALS patients with coexisting AIDs and those without were compared to those without. Results in our database include 26 ALS patients with AIDs. ALS patients with AIDs had longer onset and poorer respiratory function than controls compared to controls. We found no differences in overall survival between ALS patients with and without AIDs before and after matching. Patients with coexisting ALS and AIDs had older onset age and poorer respiratory function, but overall survival was similar to those with pure ALS.

Source link: https://doi.org/10.1371/journal.pone.0266529


Towards Understanding the Relationship Between ER Stress and Unfolded Protein Response in Amyotrophic Lateral Sclerosis

Cells developed resistance to ER stress by the introduction of an integrated signal transduction pathway known as the unfolded protein response. It has long been known that ER stress is a key contributor to ALS pathogenesis through proteostasis dysregulation. The intricate interplay between ER stress and TDP-43 pathology in ALS and TDP-43 pathology will be explored in this mini-review by taking into account the results from in vitro and in vivo models of ALS. We also discuss therapeutic options to reduce ER stress and UPR signaling components that have contrasting effects on ALS pathogenesis.

Source link: https://doi.org/10.3389/fnagi.2022.892518

* 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