Abstract
Amyotrophic lateral sclerosis (ALS) is a non-demyelinating neurodegenerative disease in adults with motor disorders. Two forms exist: a sporadic form (90% of cases) and a family form due to mutations in more than 20 genes including the Superoxide dismutase 1, TAR DNA Binding Protein, Fused in Sarcoma, chromosome 9 open reading frame 72 and VAPB genes. The mechanisms associated with this pathology are beginning to be known: oxidative stress, glutamate excitotoxicity, protein aggregation, reticulum endoplasmic stress, neuroinflammation, alteration of RNA metabolism. In various neurodegenerative diseases, such as Alzheimer’s disease or multiple sclerosis, the involvement of lipids is increasingly suggested based on lipid metabolism modifications. With regard to ALS, research has also focused on the possible involvement of lipids. Lipid involvement was suggested for clinical arguments where changes in cholesterol and LDL/HDL levels were reported with, however, differences in positivity between studies. Since lipids are involved in the membrane structure and certain signaling pathways, it may be considered to look for oxysterols, mainly 25-hydroxycholesterol and its metabolites involved in immune response, or phytosterols to find suitable biomarkers for this pathology.
Highlights
Neurodegenerative diseases represent a major public health issue since many of them affect all age groups
An American study, cross-checking the results of ten studies, showed that there were 223,000 people affected by amyotrophic lateral sclerosis (ALS) worldwide but that this figure would increase by 69% in 2040, mainly due to the aging of the Oxysterols, Phytosterols and Amyotrophic Lateral Sclerosis population
The evidence of the involvement of this gene has led to the establishment of an animal model for studying the disease, which has shown the involvement of different mechanisms that can participate in the degeneration of motor neurons such as oxidative stress, protein aggregates, mitochondrial defects, and glutamate excitotoxicity (Gurney, 1994; Ripps et al, 1995)
Summary
Neurodegenerative diseases represent a major public health issue since many of them affect all age groups. Neurodegenerative diseases are classified into two subfamilies: demyelinating neurodegenerative diseases [such as multiple sclerosis (MS), peroxisomal leukodystrophies (X-ALD)] and non-demyelinating neurodegenerative diseases [including Alzheimer’s disease (AD), Parkinson’s disease (PD), Huntington’s disease, Niemann–Pick disease, amyotrophic lateral sclerosis (ALS)]. These diseases are characterized by disorders of brain functions involving one or more of these mechanisms: oxidative stress, inflammation and cell death. Oxysterols, Phytosterols and Amyotrophic Lateral Sclerosis population These studies underline the fact that there is a need to increase knowledge about this disease, such as the identification of early biomarkers of the disease or patient follow-up, which will improve patient care. One study found that a combination of riluzole and vitamin E was effective in slowing the progression of the disease but had no effect on survival (Desnuelle et al, 2001)
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