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- W2131002806 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." @default.
- W2131002806 created "2016-06-24" @default.
- W2131002806 creator A5021789757 @default.
- W2131002806 creator A5046468115 @default.
- W2131002806 creator A5047881276 @default.
- W2131002806 creator A5072337975 @default.
- W2131002806 creator A5090762332 @default.
- W2131002806 date "2014-01-01" @default.
- W2131002806 modified "2023-10-01" @default.
- W2131002806 title "A plural role for lipids in motor neuron diseases: energy, signaling and structure" @default.
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