Low fat diets increase survival of a mouse model of spinal muscular atrophy

Ann Clin Transl Neurol. 2019 Nov;6(11):2340-2346. doi: 10.1002/acn3.50920. Epub 2019 Oct 13.


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.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Diet, Fat-Restricted*
  • Disease Models, Animal
  • Mice
  • Mice, Inbred C57BL
  • Muscular Atrophy, Spinal* / pathology
  • Muscular Atrophy, Spinal* / physiopathology