Dominant mutations in an antioxidant enzyme superoxide dismutase-1 (SOD1) cause amyotrophic lateral sclerosis (ALS), an adult-onset neurodegenerative disease characterized by loss of motor neurons. Oxidative stress has also been linked to many of the neurodegenerative diseases and is likely a central mechanism of motor neuron death in ALS. Astrocytes derived from mutant SOD1 mouse models or patients play a significant role in the degeneration of spinal motor neurons in ALS through a non-cell-autonomous process. Here we characterize the neuroprotective effects and mechanisms of urate (a.k.a. uric acid), a major endogenous antioxidant and a biomarker of favorable ALS progression rates, in a cellular model of ALS. Our results demonstrate a significant protective effect of urate against motor neuron injury evoked by mutant astrocytes derived from SOD1 mice or hydrogen peroxide induced oxidative stress. Overall, these results implicate astrocyte dependent protective effect of urate in a cellular model of ALS. These findings together with our biomarker data may advance novel targets for treating motor neuron disease.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.mcn.2018.06.002 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Neural Mass Modeling in the Cortical Motor Area and the Mechanism of Alpha Rhythm Changes.
Sensors (Basel)
December 2024
Department of Neurosurgery, Xuanwu Hospital, Capital Medical University, Beijing 100032, China.
Investigating the physiological mechanisms in the motor cortex during rehabilitation exercises is crucial for assessing stroke patients' progress. This study developed a single-channel Jansen neural mass model to explore the relationship between model parameters and motor cortex mechanisms. Firstly, EEG signals were recorded from 11 healthy participants under 20%, 40%, and 60% maximum voluntary contraction, and alpha rhythm power spectral density characteristics were extracted using the Welch power spectrum method.
View Article and Find Full Text PDFFucosidosis: A Review of a Rare Disease.
Int J Mol Sci
January 2025
Department of Molecular Biology and Genetics, Çanakkale Onsekiz Mart University, Çanakkale 17100, Turkey.
Fucosidosis is a rare lysosomal storage disease caused by α-L-fucosidase deficiency following a mutation in the gene. This enzyme is responsible for breaking down fucose-containing glycoproteins, glycolipids, and oligosaccharides within the lysosome. Mutations in result in either reduced enzyme activity or complete loss of function, leading to the accumulation of fucose-rich substrates in lysosomes.
View Article and Find Full Text PDFParkinson's Disease Prediction: An Attention-Based Multimodal Fusion Framework Using Handwriting and Clinical Data.
Diagnostics (Basel)
December 2024
Department of Information Technology, Aylol University College, Yarim 547, Yemen.
Background: Neurodegenerative diseases (NGD) encompass a range of progressive neurological conditions, such as Alzheimer's disease (AD) and Parkinson's disease (PD), characterised by the gradual deterioration of neuronal structure and function. This degeneration manifests as cognitive decline, movement impairment, and dementia. Our focus in this investigation is on PD, a neurodegenerative disorder characterized by the loss of dopamine-producing neurons in the brain, leading to motor disturbances.
View Article and Find Full Text PDFIdentifying novel response markers for spinal muscular atrophy revealed by targeted proteomics following gene therapy.
Gene Ther
January 2025
Departments of Pediatrics and Neurology, Emory University, Atlanta, 30322, Georgia.
Spinal muscular atrophy (SMA) is a progressive disease that affects motor neurons, with symptoms usually starting in infancy or early childhood. Recent breakthroughs in treatments targeting SMA have improved both lifespan and quality of life for infants and children with the disease. Given the impact of these treatments, it is essential to develop methods for managing treatment-induced changes in disease characteristics.
View Article and Find Full Text PDFMachine learning identified novel players in lipid metabolism, endosomal trafficking, and iron metabolism of the ALS spinal cord.
Sci Rep
January 2025
Graduate Institute of Integrated Medicine, College of Chinese Medicine, China Medical University, Taichung, 40402, Taiwan.
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease affecting motor neurons. Although genes causing familial cases have been identified, those of sporadic ALS, which occupies the majority of patients, are still elusive. In this study, we adopted machine learning to build binary classifiers based on the New York Genome Center (NYGC) ALS Consortium's RNA-seq data of the postmortem spinal cord of ALS and non-neurological disease control.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!