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Low fat diets increase survival of a mouse model of spinal muscular atrophy.
Authors Deguise MO, Chehade L, Tierney A, Beauvais A, Kothary R
Submitted By Submitted Externally on 10/17/2019
Status Published
Journal Annals of clinical and translational neurology
Year 2019
Date Published 10/1/2019
Volume : Pages Not Specified : Not Specified
PubMed Reference 31608604
Abstract 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.


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