Redox Regulation and Misfolding of SOD1: Therapeutic Strategies for Amyotrophic Lateral Sclerosis

2016 ◽  
pp. 605-626
Author(s):  
Wouter Hubens ◽  
Ayako Okado-Matsumoto
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Redox Regulation ◽  
Therapeutic Strategies ◽  
Lateral Sclerosis

Plant-Derived Natural Compounds for the treatment of Amyotrophic Lateral Sclerosis: An Update

2021 ◽  
Vol 19 ◽  
Author(s):  
Roohi Mohi-ud-din ◽  
Reyaz Hassan Mir ◽  
Abdul Jalil Shah ◽  
Saba Sabreen ◽  
Taha Umair Wani ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Amyotrophic Lateral Sclerosis ◽  
Adverse Effects ◽  
Therapeutic Strategies ◽  
Structure Activity Relationships ◽  
Complex Genetics ◽  
Natural Agents ◽  
Structure Activity ◽  
Preclinical And Clinical Studies ◽  
Lateral Sclerosis

Background: Amyotrophic lateral sclerosis (ALS) is a motor neuron disease (MND) that typically causes death within 3-5 years after diagnosis. Regardless of the substantial scientific knowledge accrued from more than a century ago, truly effective therapeutic strategies remain distant. Various conventional drugs are being used but are having several adverse effects. Objective/Aim: The current study aims to thoroughly review plant-derived compounds with well-defined ALS activities and their structure-activity relationships. Moreover, the review also focuses on complex genetics, clinical trials, and the use of natural products that might decrypt the future and novel therapeutics in ALS. Methods: The collection of data for the compilation of this review work was searched in PubMed Scopus, Google Scholar, and Science Direct. Results: Results showed that phytochemicals like-Ginkgolides, Protopanaxatriol, Genistein, epigallocatechingallate, resveratrol, cassoside, and others possess Amyotrophic lateral sclerosis (ALS) activity by various mechanisms. Conclusion: These plant-derived compounds may be considered as supplements to conventional (ALS). Moreover, further preclinical and clinical studies are required to understand the structure-activity relationships, metabolism, absorption, and mechanisms of plant-derived natural agents.

scholarly journals Amyotrophic Lateral Sclerosis: A Focus on Disease Progression

2014 ◽  
Vol 2014 ◽  
pp. 1-12 ◽  
Author(s):  
Ana C. Calvo ◽  
Raquel Manzano ◽  
Deise M. F. Mendonça ◽  
María J. Muñoz ◽  
Pilar Zaragoza ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Molecular Targets ◽  
Therapeutic Strategies ◽  
Molecular Pathways ◽  
Neuron Death ◽  
Parkinson’S Diseases ◽  
Wide Range ◽  
Motor Neuron Death ◽  
Lateral Sclerosis ◽  
Potential Biomarkers

Since amyotrophic lateral sclerosis (ALS) was discovered and described in 1869 as a neurodegenerative disease in which motor neuron death is induced, a wide range of biomarkers have been selected to identify therapeutic targets. ALS shares altered molecular pathways with other neurodegenerative diseases, such as Alzheimer’s, Huntington’s, and Parkinson’s diseases. However, the molecular targets that directly influence its aggressive nature remain unknown. What is the first link in the neurodegenerative chain of ALS that makes this disease so peculiar? In this review, we will discuss the progression of the disease from the viewpoint of the potential biomarkers described to date in human and animal model samples. Finally, we will consider potential therapeutic strategies for ALS treatment and future, innovative perspectives.

scholarly journals Amyotrophic Lateral Sclerosis and Novel Therapeutic Strategies

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Brett Morrison ◽  
Kenneth Hensley ◽  
Erik P. Pioro ◽  
Susanne Petri ◽  
Mahmoud Kiaei
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Therapeutic Strategies ◽  
Novel Therapeutic Strategies ◽  
Lateral Sclerosis ◽  
Novel Therapeutic

scholarly journals Existing and Emerging Metabolomic Tools for ALS Research

Genes ◽  
2019 ◽  
Vol 10 (12) ◽  
pp. 1011 ◽  
Author(s):  
Christine Germeys ◽  
Tijs Vandoorne ◽  
Valérie Bercier ◽  
Ludo Van Den Bosch
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Energy Metabolism ◽  
Motor Neurons ◽  
Therapeutic Strategies ◽  
Metabolic State ◽  
Advanced Technologies ◽  
Novel Therapeutic Strategies ◽  
Lateral Sclerosis ◽  
Novel Therapeutic

Growing evidence suggests that aberrant energy metabolism could play an important role in the pathogenesis of amyotrophic lateral sclerosis (ALS). Despite this, studies applying advanced technologies to investigate energy metabolism in ALS remain scarce. The rapidly growing field of metabolomics offers exciting new possibilities for ALS research. Here, we review existing and emerging metabolomic tools that could be used to further investigate the role of metabolism in ALS. A better understanding of the metabolic state of motor neurons and their surrounding cells could hopefully result in novel therapeutic strategies.

scholarly journals Redox Regulation in Amyotrophic Lateral Sclerosis

2013 ◽  
Vol 2013 ◽  
pp. 1-12 ◽  
Author(s):  
Sonam Parakh ◽  
Damian M. Spencer ◽  
Mark A. Halloran ◽  
Kai Y. Soo ◽  
Julie D. Atkin
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neurons ◽  
Protein Misfolding ◽  
Redox Regulation ◽  
Stress Protein ◽  
Glutamate Excitotoxicity ◽  
Chaperone Protein ◽  
Cellular Abnormalities ◽  
Underlying Mechanisms ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that results from the death of upper and lower motor neurons. Due to a lack of effective treatment, it is imperative to understand the underlying mechanisms and processes involved in disease progression. Regulations in cellular reduction/oxidation (redox) processes are being increasingly implicated in disease. Here we discuss the possible involvement of redox dysregulation in the pathophysiology of ALS, either as a cause of cellular abnormalities or a consequence. We focus on its possible role in oxidative stress, protein misfolding, glutamate excitotoxicity, lipid peroxidation and cholesterol esterification, mitochondrial dysfunction, impaired axonal transport and neurofilament aggregation, autophagic stress, and endoplasmic reticulum (ER) stress. We also speculate that an ER chaperone protein disulphide isomerase (PDI) could play a key role in this dysregulation. PDI is essential for normal protein folding by oxidation and reduction of disulphide bonds, and hence any disruption to this process may have consequences for motor neurons. Addressing the mechanism underlying redox regulation and dysregulation may therefore help to unravel the molecular mechanism involved in ALS.

scholarly journals Therapeutic strategies for C9orf72 amyotrophic lateral sclerosis and frontotemporal dementia

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Guillaume M. Hautbergue ◽  
John D. Cleary ◽  
Shu Guo ◽  
Laura P.W. Ranum
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Frontotemporal Dementia ◽  
Therapeutic Strategies ◽  
Lateral Sclerosis

scholarly journals Molecular mechanisms and therapeutic strategies in amyotrophic lateral sclerosis caused by C9orf72 mutations

The Lancet ◽  
2016 ◽  
Vol 387 ◽  
pp. S13
Author(s):  
Rubika Balendra ◽  
Sarah Mizielinska ◽  
Thomas Moens ◽  
Teresa Niccoli ◽  
Charlotte Ridler ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Molecular Mechanisms ◽  
Therapeutic Strategies ◽  
Lateral Sclerosis
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