Abstract
Motor neuron diseases (MNDs) are characterized by selective death of motor neurons and include mainly adult-onset amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA). Neurodegeneration is not the single pathogenic event occurring during disease progression. There are multiple lines of evidence for the existence of defects in lipid metabolism at peripheral level. For instance, hypermetabolism is well characterized in ALS, and dyslipidemia correlates with better prognosis in patients. Lipid metabolism plays also a role in other MNDs. In SMA, misuse of lipids as energetic nutrients is described in patients and in related animal models. The composition of structural lipids in the central nervous system is modified, with repercussion on membrane fluidity and on cell signaling mediated by bioactive lipids. Here, we review the main epidemiologic and mechanistic findings that link alterations of lipid metabolism and motor neuron degeneration, and we discuss the rationale of targeting these modifications for therapeutic management of MNDs.
Highlights
Motor neuron diseases (MNDs) are a group of incurable neurological disorders caused by the selective degeneration of motor neurons
Several mutations are associated with Amyotrophic lateral sclerosis (ALS), in particular in genes encoding superoxide dismutase 1 (SOD-1), TAR DNA binding protein of 43-kDa (TDP-43), fused in sarcoma (FUS) and chromosome 9 open reading frame 72 (C9ORF72) (Rosen et al, 1993; Mackenzie et al, 2007; Deng et al, 2010; Laaksovirta et al, 2010; Shatunov et al, 2010)
Spinal bulbar muscular atrophy (SBMA) is characterized by progressive muscle atrophy and degeneration of lower motor neurons in the brain stem and spinal cord
Summary
A plural role for lipids in motor neuron diseases: energy, signaling and structure. Reviewed by: Ricardo Tapia, Universidad Nacional Autónoma de México, Mexico Luis B. Tovar-y-Romo, Universidad Nacional Autónoma de México, Mexico. Motor neuron diseases (MNDs) are characterized by selective death of motor neurons and include mainly adult-onset amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA). Lipid metabolism plays a role in other MNDs. In SMA, misuse of lipids as energetic nutrients is described in patients and in related animal models. The composition of structural lipids in the central nervous system is modified, with repercussion on membrane fluidity and on cell signaling mediated by bioactive lipids. We review the main epidemiologic and mechanistic findings that link alterations of lipid metabolism and motor neuron degeneration, and we discuss the rationale of targeting these modifications for therapeutic management of MNDs
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