Abstract
Amyotrophic lateral sclerosis (ALS) is a fatal degenerative disease primarily characterized by the selective loss of upper and lower motor neurons. To date, there is still an unmet need for robust and practical biomarkers that could estimate the risk of the disease and its progression. Based on metabolic modifications observed at the level of the whole body, different classes of lipids have been proposed as potential biomarkers. This review summarizes investigations carried out over the last decade that focused on changes in three major lipid species, namely cholesterol, triglycerides and fatty acids. Despite some contradictory findings, it is becoming increasingly accepted that dyslipidemia, and related aberrant energy homeostasis, must be considered as essential components of the pathological process. Therefore, it is tempting to envisage dietary interventions as a means to counterbalance the metabolic disturbances and ameliorate the patient's quality of life.
Highlights
Reviewed by: Débora Lanznaster, INSERM U1253Imagerie et Cerveau, France Philippe Couratier, Centre Hospitalier Universitaire de Limoges, France
Levels of several oxysterol metabolites, including 27-hydroxycholesterol, 24-hydroxycholesterol esters, and 3β,7α-dihydroxycholest-5-en-26-oic acid and other related compounds, were shown to be lower in Amyotrophic lateral sclerosis (ALS) patients. These changes were detected in blood or cerebrospinal fluid, or both, and they were attributed to a deficit in the metabolism of excess cholesterol, which would result in subsequent toxicity in the brain [46, 52, 53]
Many lipid molecules have been proposed as promising biomarkers for ALS, but none of them has been translated into effective tools in clinical practice
Summary
Reviewed by: Débora Lanznaster, INSERM U1253Imagerie et Cerveau (iBrain), France Philippe Couratier, Centre Hospitalier Universitaire de Limoges, France. Amyotrophic lateral sclerosis (ALS) is a fatal degenerative disease primarily characterized by the selective loss of upper and lower motor neurons. There is still an unmet need for robust and practical biomarkers that could estimate the risk of the disease and its progression. Based on metabolic modifications observed at the level of the whole body, different classes of lipids have been proposed as potential biomarkers. This review summarizes investigations carried out over the last decade that focused on changes in three major lipid species, namely cholesterol, triglycerides and fatty acids. It is tempting to envisage dietary interventions as a means to counterbalance the metabolic disturbances and ameliorate the patient’s quality of life
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