2007/04/17 by Anissa Fergani, A. Fergani, H. Oudart +15 · 117 citations
Biochemistry, Genetics and Molecular Biology · Chemistry · Medicine · Neuroscience · #Amyotrophic Lateral Sclerosis Research #Amyotrophic lateral sclerosis #Atrophy #Biology #Chemistry #Cholesterol #Disease #Endocrinology #Insulin #Internal medicine #Lipid metabolism #Medicine #Motor neuron #Neurological diseases and metabolism #Neuroprotection #Parkinson's Disease Mechanisms and Treatments #Postprandial #Triglyceride #q-bio.PE
paper · pdf · doi:10.1194/jlr.m700017-jlr200
published in Journal of Lipid Research 48(7), 1571-1580 (Elsevier BV)
openalex publication_date 2007/04/17 · arxiv created 2007/07/09 · arxiv updated 2009/12/01 · openalex created_date 2025/10/10 · openalex updated_date 2026/08/05
Amyotrophic lateral sclerosis (ALS) is the most common adult motor neuron disease, causing motor neuron degeneration, muscle atrophy, paralysis, and death. Despite this degenerative process, a stable hypermetabolic state has been observed in a large subset of patients. Mice expressing a mutant form of Cu/Zn-superoxide dismutase (mSOD1 mice) constitute an animal model of ALS that, like patients, exhibits unexpectedly increased energy expenditure. Counterbalancing for this increase with a high-fat diet extends lifespan and prevents motor neuron loss. Here, we investigated whether lipid metabolism is defective in this animal model. Hepatic lipid metabolism was roughly normal, whereas gastrointestinal absorption of lipids as well as peripheral clearance of triglyceride-rich lipoproteins were markedly increased, leading to decreased postprandial lipidemia. This defect was corrected by the high-fat regimen that typically induces neuroprotection in these animals. Together, our findings show that energy metabolism in mSOD1 mice shifts toward an increase in the peripheral use of lipids. This metabolic shift probably accounts for the protective effect of dietary lipids in this model.