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Amyotrophic Lateral Sclerosis: Molecular Mechanisms, Biomarkers, and Therapeutic Strategies

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8300638/
Go to: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with the progressive loss of motor neurons, leading to a fatal paralysis. According to whether there is a family history of ALS, ALS can be roughly divided into two types: familial and sporadic. Despite decades of research, the pathogenesis of ALS is still unelucidated.

Disease Mechanisms | The ALS Association

https://www.als.org/research/als-research-topics/disease-mechanisms
Disease mechanisms (i.e. the process of ALS disease) help define causes of ALS. Researchers from all over the world are working to better understand ALS disease mechanisms. ALS is a heterogeneous disease, meaning that there are many diverse ways that disease can occur.

Pathophysiology and Diagnosis of ALS: Insights from Advances in

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6600525/
1. Introduction. Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder of the human motor system, clinically characterized by dysfunction of the upper and lower motor neurons, which forms the basis of diagnosis [1,2,3].While disease onset is typically focal involving upper or lower limbs, bulbar or respiratory regions, the ensuing progressive course affects

Molecular and Cellular Mechanisms Affected in ALS - PMC

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7564998/
Amyotrophic lateral sclerosis (ALS) is a terminal late-onset condition characterized by the loss of upper and lower motor neurons. Mutations in more than 30 genes are associated to the disease, but these explain only ~20% of cases. The molecular functions of these genes implicate a wide range of cellular processes in ALS pathology, a cohesive

Amyotrophic lateral sclerosis: a neurodegenerative disorder ... - Nature

https://www.nature.com/articles/s41573-022-00612-2
Amyotrophic lateral sclerosis (ALS) is a devastating disease caused by degeneration of motor neurons. As with all major neurodegenerative disorders, development of disease-modifying therapies has

Amyotrophic lateral sclerosis | Nature Reviews Disease Primers

https://www.nature.com/articles/nrdp201771
Histopathology. Although the fundamental pathophysiological mechanisms underlying ALS are not well understood, the neuropathological hallmark of disease is the aggregation and accumulation of

ALS Genetics, Mechanisms, and Therapeutics: Where Are We Now?

https://www.frontiersin.org/journals/neuroscience/articles/10.3389/fnins.2019.01310/full
ALS is the most common adult motor neuron disease with an incidence of 2 per 100,000 and prevalence of 5.4 per 100,000 individuals ( ChiĆ² et al., 2013 ). Current treatment options are based on symptom management and respiratory support with the only approved medications in widespread use, Riluzole and Edaravone, providing only modest benefits

Decoding ALS: from genes to mechanism | Nature

https://www.nature.com/articles/nature20413
Amyotrophic lateral sclerosis (ALS) is a progressive and uniformly fatal neurodegenerative disease. ... Substantial evidence has also accrued to implicate a third disease mechanism in C9 ALS-FTD

Pathophysiology and Diagnosis of ALS: Insights from Advances in

https://pubmed.ncbi.nlm.nih.gov/31185581/
Amyotrophic lateral sclerosis (ALS) is a rapidly progressive and fatal neurodegenerative disorder of the motor neurons, characterized by focal onset of muscle weakness and incessant disease progression. ... (TMS) techniques have established cortical hyperexcitability as an important pathogenic mechanism in ALS, correlating with

Mechanism of Disease: ALS - YouTube

https://www.youtube.com/watch?v=oNsP67M57tM
Amyotrophic lateral sclerosis (ALS) is a fatal, adult-onset neurodegenerative disorder. Symptoms include progressive muscle weakness, paralysis and respirato

Disease Mechanisms | ALS Northwest

https://alsoregon.org/research/understanding_als_research/disease-mechanisms/
Understanding ALS disease mechanisms is crucial for defining its causes and developing therapeutic targets. Given the heterogeneity of ALS, multiple treatment approaches targeting various disease processes may be beneficial for individuals living with the disease. Researchers worldwide are actively working to uncover these mechanisms and direct

Amyotrophic Lateral Sclerosis (ALS) | National Institute of

https://www.ninds.nih.gov/health-information/disorders/amyotrophic-lateral-sclerosis-als
Amyotrophic lateral sclerosis (ALS), formerly known as Lou Gehrig's disease, is a neurological disorder that affects motor neurons, the nerve cells in the brain and spinal cord that control voluntary muscle movement and breathing. As motor neurons degenerate and die, they stop sending messages to the muscles, which causes the muscles to weaken

Amyotrophic Lateral Sclerosis: Autoimmune Pathogenic Mechanisms

https://pubmed.ncbi.nlm.nih.gov/31507117/
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the progressive death of motor neurons leading to fatal paralysis. The causes of ALS remain unknown; however, evidence supports the presence of autoimmune mechanisms contributing to pathogenesis. Although several en

Amyotrophic Lateral Sclerosis: Molecular Mechanisms, Biomarkers, and

https://pubmed.ncbi.nlm.nih.gov/34202494/
Abstract. Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with the progressive loss of motor neurons, leading to a fatal paralysis. According to whether there is a family history of ALS, ALS can be roughly divided into two types: familial and sporadic. Despite decades of research, the pathogenesis of ALS is still unelucidated.

Understanding 'disease mechanisms' of ALS | ScienceDaily

https://www.sciencedaily.com/releases/2018/03/180308155255.htm
Understanding 'disease mechanisms' of ALS Date: March 8, 2018 Source: Syracuse University Summary: Researchers are making strides in understanding the disease mechanism of amyotrophic lateral

Amyotrophic lateral sclerosis: mechanisms and therapeutics in the

https://www.nature.com/articles/nrneurol.2015.57
Amyotrophic lateral sclerosis (ALS) involves a complex interaction between genetic susceptibility and environmental insults. In this Review, Paez-Colasante and colleagues discuss various disease

Pathological mechanisms of amyotrophic lateral sclerosis - PMC

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10749610/
Proposed Pathogenic Mechanisms of Amyotrophic Lateral Sclerosis. Although the specific pathogenic mechanisms of ALS remain unresolved after many years of research, the pathophysiological mechanisms of the disease may be due to a combination of mechanisms mediated via complicated interactions among molecular and genetic pathways rather than a single factor.

Amyotrophic Lateral Sclerosis [ALS] | Mechanism of Neuron Death

https://www.youtube.com/watch?v=dLHS8XzGO6g
READ MORE BELOW!In this video, we explore the basic biochemical mechanism of neuronal death in amyotrophic lateral sclerosis or ALS. ALS Clinical Presentatio

Scientists identify genes linked to brain cell loss in ALS

https://www.nih.gov/news-events/news-releases/scientists-identify-genes-linked-brain-cell-loss-als
What In a small study, researchers have discovered how a set of genes could cause neurons to die in sporadic amyotrophic lateral sclerosis (ALS).The results, published in Nature Aging, provide insight into the root causes of ALS and may lead to new ways to halt disease progression.The study was funded by the National Institutes of Health (NIH). ALS is a progressive neurological disorder that

Finding the master switch for ALS | The Neuro - McGill University

https://www.mcgill.ca/neuro/article/research/finding-master-switch-als
Robitaille's lab is focussed on finding ways to repair the neuromuscular junction. Damage to this junction is known to become occur very early in the disease and is a common factor in all ALS cases. According to the ALS Society of Canada, each year approximately 1,000 Canadians die from the disease.

Pathological mechanisms of amyotrophic lateral Sclerosis

https://pubmed.ncbi.nlm.nih.gov/37862206/
According to current research, amyotrophic lateral sclerosis is likely not due to a single factor but rather to a combination of mechanisms mediated by complex interactions between molecular and genetic pathways. The progression of the disease involves multiple cellular processes and the interaction between different complex mechanisms makes it

Mechanism of motoneuronal and pyramidal cell death in ... - Nature

https://www.nature.com/articles/s41420-024-02055-7
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder, characterized by the selective loss of bulbar and spinal cord motoneurons and pyramidal Betz cells in the motor cortex.

New biomarker may help detect presymptomatic ALS and FTD

https://www.nia.nih.gov/news/new-biomarker-may-help-detect-presymptomatic-als-and-ftd
A newly discovered fluid biomarker may help identify amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) before symptoms begin. In a study published in Nature Medicine, NIA researchers demonstrated the ability of their test to detect molecular signs of ALS and FTD. The findings suggest that the method could potentially be used

Mechanisms of neurodegeneration in amyotrophic lateral sclerosis

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1187128/
This review discusses the major mechanisms of neurodegeneration in ALS, such as oxidative stress, glutaminergic excitotoxicity, damage to vital organelles, and aberrant protein aggregation. Keywords: amyotrophic lateral sclerosis, motor neurone disease, neurodegeneration. Amyotrophic lateral sclerosis (ALS) is the most common variant of motor

Scientists identify genes linked to brain cell loss in ALS

https://www.ninds.nih.gov/news-events/news/press-releases/scientists-identify-genes-linked-brain-cell-loss-als
NIH-funded study shines light on disease mechanisms, ... In a small study, researchers have discovered how a set of genes could cause neurons to die in sporadic amyotrophic lateral sclerosis (ALS). The results, published in Nature Aging, provide insight into the root causes of ALS and may lead to new ways to halt disease progression.

ALS: Newly discovered subtypes and sex differences create new

https://www.news-medical.net/news/20240621/ALS-Newly-discovered-subtypes-and-sex-differences-create-new-approaches-for-drugs.aspx
Amyotrophic lateral sclerosis, ALS for short, is a mysterious neurodegenerative disease that is almost always fatal. A consortium of researchers led by the Technical University of Munich (TUM) has

Amyotrophic lateral sclerosis: translating genetic discoveries into

https://www.nature.com/articles/s41576-023-00592-y
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that primarily affects motor neurons, leading to progressive muscle weakness and death from respiratory failure within 2-5

Most SOD1 mutations are pathogenic, and their identification can lead

https://jnnp.bmj.com/content/early/2024/06/25/jnnp-2024-333939
Coding mutations in the gene encoding SOD1 were found to co-segregate with disease in fALS cases in 1993, and since then, over 230 mutations in SOD1 have been reported in ALS showing heterogeneity in disease penetrance and phenotype. The suspected mechanism by which the SOD1 mutated protein causes ALS is by gain of a novel toxic function which includes the generation of prion-like SOD1 species.

The Complex Molecular Biology of Amyotrophic Lateral Sclerosis (ALS)

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3605887/
The sporadic disease differs little clinically from SOD1-related FALS, leading to the widespread supposition that all cases of ALS share some common mechanism(s) of pathology. 2,45,46 In reviewing the proposed molecular bases of ALS, we focus on the contribution of SOD1, a well-studied cause of ALS that may exhibit pathogenic mechanisms common

Whole-Genome Sequencing Identified a Novel Mutation in the N ... - PubMed

https://pubmed.ncbi.nlm.nih.gov/38927616/
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder characterized by progressive damage to both upper and lower motor neurons. Genetic factors are known to play a crucial role in ALS, as genetic studies not only advance our comprehension of disease mechanisms but also help unravel the complex phenotypes exhibited by