Low fat diets increase survival of a mouse model of spinal muscular atrophy.
Marc-Olivier DeguiseLucia ChehadeAlexandra TierneyAriane BeauvaisRashmi KotharyPublished in: Annals of clinical and translational neurology (2019)
Spinal muscular atrophy (SMA) is a neuromuscular disorder leading to paralysis and death. Recent evidence shows increased susceptibility to dyslipidemia and liver steatosis in patients. Here, we provide evidence that low fat diets nearly double survival in Smn2B/- mice, a model for SMA, independent of changes in SMN levels, liver steatosis, or enhanced hepatic functions. Liver damage and ketone levels were reduced, implying a lower reliance on fatty acid oxidation. This preclinical proof of concept study provides grounds for controlled clinical investigation of dietary needs and offers evidence to inform nutritional guidelines specific to SMA.
Keyphrases
- fatty acid
- high fat diet induced
- mouse model
- end stage renal disease
- adipose tissue
- insulin resistance
- high fat diet
- ejection fraction
- weight loss
- newly diagnosed
- chronic kidney disease
- oxidative stress
- prognostic factors
- cell therapy
- clinical practice
- stem cells
- peritoneal dialysis
- type diabetes
- metabolic syndrome
- patient reported