期刊
ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY
卷 6, 期 11, 页码 2340-2346出版社
WILEY
DOI: 10.1002/acn3.50920
关键词
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资金
- Cure SMA/Families of SMA Canada
- Muscular Dystrophy Association (USA) [575466]
- Canadian Institutes of Health Research (CIHR) [PJT-156379]
- National Institutes of Health [DK59637]
- Frederick Banting and Charles Best CIHR Doctoral Research Award
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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