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

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 Smn(2B/−) mice, a model for SMA, independen...

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Autores principales: Deguise, Marc‐Olivier, Chehade, Lucia, Tierney, Alexandra, Beauvais, Ariane, Kothary, Rashmi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2019
Materias:
Brief Communications
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6856606/
https://www.ncbi.nlm.nih.gov/pubmed/31608604
http://dx.doi.org/10.1002/acn3.50920
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author Deguise, Marc‐Olivier
Chehade, Lucia
Tierney, Alexandra
Beauvais, Ariane
Kothary, Rashmi
author_facet Deguise, Marc‐Olivier
Chehade, Lucia
Tierney, Alexandra
Beauvais, Ariane
Kothary, Rashmi
author_sort Deguise, Marc‐Olivier
collection PubMed
description 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 Smn(2B/−) 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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spelling pubmed-68566062019-12-12 Low fat diets increase survival of a mouse model of spinal muscular atrophy Deguise, Marc‐Olivier Chehade, Lucia Tierney, Alexandra Beauvais, Ariane Kothary, Rashmi Ann Clin Transl Neurol Brief Communications 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 Smn(2B/−) 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. John Wiley and Sons Inc. 2019-10-13 /pmc/articles/PMC6856606/ /pubmed/31608604 http://dx.doi.org/10.1002/acn3.50920 Text en © 2019 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals, Inc on behalf of American Neurological Association. This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Brief Communications
Deguise, Marc‐Olivier
Chehade, Lucia
Tierney, Alexandra
Beauvais, Ariane
Kothary, Rashmi
Low fat diets increase survival of a mouse model of spinal muscular atrophy
title Low fat diets increase survival of a mouse model of spinal muscular atrophy
title_full Low fat diets increase survival of a mouse model of spinal muscular atrophy
title_fullStr Low fat diets increase survival of a mouse model of spinal muscular atrophy
title_full_unstemmed Low fat diets increase survival of a mouse model of spinal muscular atrophy
title_short Low fat diets increase survival of a mouse model of spinal muscular atrophy
title_sort low fat diets increase survival of a mouse model of spinal muscular atrophy
topic Brief Communications
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6856606/
https://www.ncbi.nlm.nih.gov/pubmed/31608604
http://dx.doi.org/10.1002/acn3.50920
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