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Amyotrophic Lateral Sclerosis - DOAJ

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Last Updated: 27 October 2022

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Discovery of Mitophagy Inhibitors with Therapeutic Potential in Different Familial Amyotrophic Lateral Sclerosis Mutations

We recently observed an increase in autophagy flux in lymphoblasts from ALS patients with a SOD1 mutation. We discovered a new mitophagy inhibitor, the small molecule IGS2. 7 from the MBC library, here, using a phenotypic mitophagy assay. Interestingly, autophagy is returned to normal levels following the therapy of several cell and in vivo models of ALS with mutations on SOD1 and TARDBP with this inhibitor. These findings point to a new therapeutic strategy for familial ALS patients, and IGS2. 7 in particular.

Source link: https://doi.org/10.3390/ijms232012676


Advances on Cellular Clonotypic Immunity in Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (PE) is a fatal neuromuscular disorder characterized by the degeneration of the upper and lower motor neurons in the cortex and spinal cord. Though the pathogenesis of ALS remains unclear, evidence regarding the clonypic immune system's function is increasing. An attempt is made in the present review to reveal the true role of cellular clonotypic immunity in ALS by integrating new clinical findings and experimental findings.

Source link: https://doi.org/10.3390/brainsci12101412


Correlation between Retinal Vascularization and Disease Aggressiveness in Amyotrophic Lateral Sclerosis

In ALS patients, the aim of the present research was to quantitatively investigate retinal layers and vascularization by means of spectral-domain and optical coherence tomography angiography. According to the disease progression rate, ALS patients were divided into three categories: slow progressors, intermediate progressors, and rapid progressors. We analyzed Subfoveal choroidal thickness, ganglion cell complex, and retinal nerve fiber layer for SD-OCT. The SD-OCT exam revealed no significant differences in GCC and RNFL thickness between patients and controls and among the three ALS groups. The SFCT was statistically higher in patients compared to controls; more importantly, it was thicker in patients with slow and intermediate disease progression than in those with rapid disease progression. ALS has been shown by this study that there is a possible connection between choroidal thickness and disease activity.

Source link: https://doi.org/10.3390/biomedicines10102390


Study on the Diagnostic Value of Neuroelectrophysiological Examination in Patients with Amyotrophic Lateral Sclerosis

Our aim was to determine the diagnostic value of neuroelectrophysiological examination in patients with amyotrophic lateral sclerosis. There was a statistical discrepation between clinically reported ALS and the proposed ALS and potential ALS results. The abnormality of suspected ALS was particularly apparent, and the overall abnormal rate of EMG in ALS was 85 percent. There was no significant difference between clinically diagnosed ALS and suspected ALS, and there was no significant difference between medically diagnosed ALS and ALS, and there was no significant difference between clinically diagnosed ALS and probable ALS, and there was no significant difference between clinically diagnosed ALS and potential ALS, and there was no significant difference between clinically diagnosed ALS and ALS, and there was no significant difference between clinically diagnosed ALS and probable ALS, and there was no significant difference between clinically diagnosed ALS The frequency of F wave in the ALS group decreased, which may be attributed to the presence of F wave conduction pathway and the presence of reduced motor neuron involvement. Patients with clinically contaminated ALS should perform electrophysiological testing as soon as possible, which is helpful in the early diagnosis and differential diagnosis of ALS.

Source link: https://doi.org/10.1155/2022/3907751


Different observation period of exercise training in amyotrophic lateral sclerosis patients: A meta-analysis

Methods (German)Methods was a scientist who sought to determine the effect of more intensive exercise training on amyotrophic lateral sclerosis patients with amyotrophic lateral sclerosis patients. This meta-analysis was conducted using a fixed effect model or random effect model. In the short term and the medium term, the findings showed that there was statistical significance in the effects of more vigorous exercise training on amyotrophic lateral sclerosis Functional Rating Scale. In the short run, there was no significant difference between the two forces' vital function in the short run, but more research should be done in the future to determine the effects of more intense exercise training in patients with amyotrophic lateral sclerosis.

Source link: https://doi.org/10.3389/fneur.2022.986882


Synergistic association of resveratrol and histone deacetylase inhibitors as treatment in amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis is a fatal neurodegenerative disease associated with motor neuron degeneration, progressive paralysis, and eventually death. Low doses of the AMP-activated kinase-sirtuin 1 murine model of ALS can be corrected by the synergistic combination of low doses of the phospho-activated kinase -sirtuin 1 pathway activator resveratrol and histone deacetylase inhibitors MS-275 or valproate, according to new research, the pathological acetylation state of tuin 1 murine model of kana phosphorustuin 1 murine tuin 1 murine inhibitor retuin 1 murine model of calcultuin 1 murine inhibitor en acetuin 1 murine inhibitors sirtuin 1 murine sirtuin acetuin1 murine acetutuin 1 murine In this mini report, we explore the possibility of the epigenetic combination of resveratrol and HDAC inhibitors in the ALS treatment.

Source link: https://doi.org/10.3389/fphar.2022.1017364


Improved survival in amyotrophic lateral sclerosis patients following autologous bone marrow mononuclear cell therapy: a long term 10-year retrospective study

Background: Promising results from previous research using cell therapy have paved the way for a new therapy approach for amyotrophic lateral sclerosis. Objectivi: This controlled, retrospective study found that the objective of our study, which was administered to 216 patients with probable or definitive ALS, 150 in the intervention group receiving autologous BMMNCs and standard therapy, and 66 in the control group receiving only standard care, was determined to determine the influence of type of onset and hormonal status on survival rate in a large population, with 152 patients suffering from disease progression. The estimated survival duration of control group and intervention group was estimated and compared using Kaplan Meier analysis.

Source link: https://doi.org/10.26599/JNR.2021.9040010


Steady-state visual evoked potential (SSVEP)-based brain–computer interface (BCI) of Chinese speller for a patient with amyotrophic lateral sclerosis: A case report

This research demonstrated the possibility of interfacing with a patient in a lock-in state with amyotrophic lateral sclerosis and demonstrated its comitability for communication by using a steady-state visual. The findings revealed that the tested SSVEP-based BCI system was both inexpensive and efficient enough to provide daily life support to ALS patients.

Source link: https://doi.org/10.26599/JNR.2020.9040003


Clinical and genetic study of a Chinese family affected by both amyotrophic lateral sclerosis and autosomal dominant polycystic kidney disease

Amyotrophic lateral sclerosis is a progressive neurodegenerative disorder characterized by the absence of the upper and lower motor neurons from the motor cortex, brainstem, and spinal cord. Autosomal dominant polycystic kidney disease is a hereditable renal disease that ultimately results in end-stage kidney disease. We report a large family with 10 patients with ALS phenotype, 3 asymptomatic SOD1-H47R carriers, and 6 with ADPKD phenotype. We discovered a novel probable pathogenic variant in PKD1 among patients with ADPKD1 and a pathogenic variant of SOD1 in patients with ALS using whole exome sequencing.

Source link: https://doi.org/10.3389/fneur.2022.1004909


Characterization of the nigroestriatal system in a sample of patients with amyotrophic lateral sclerosis

The purpose of this paper is to demonstrate a sample of patients with clinically definitive or probable ALS who were compared with non-ALS controls. Patients with a diagnosis of ALS with fewer than ten years of duration were compared to those with a symptom of PD with less than ten years of duration, and with patients with other mobility disorders not related to neurodegenerative disorders. Patients with ALS had TRODAT measurements below that expected norms, as did 75% of the patients with no neurodegenerative disease in the control group, and 100% of the patients in the PD group. In the TRODAT results, a statistically significant difference was found between patients with ALS and the control group without neurodegenerative disease. 0. 05.

Source link: https://doi.org/10.1055/s-0042-1755282

* 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