Blunted Fat Oxidation upon Submaximal Exercise is Partially Compensated by Enhanced Glucose Metabolism in Children, Adolescents and Young Adults with Barth Syndrome

Barth syndrome (BTHS) is a rare X-linked condition resulting in abnormal mitochondria, cardioskeletal myopathy, and growth delay however; the effects of BTHS on substrate metabolism regulation and their relationships with tissue function in humans are unknown. We sought to characterize glucose and f...

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Udgivet i:J Inherit Metab Dis
Main Authors: Cade, W. Todd, Bohnert, Kathryn L., Peterson, Linda R., Patterson, Bruce W., Bittel, Adam J., Okunade, Adewole L., de las Fuentes, Lisa, Steger-May, Karen, Bashir, Adil, Schweitzer, George G., Chacko, Shaji K., Wanders, Ronald J., Pacak, Christina A., Byrne, Barry J, Reeds, Dominic N.
Format: Artigo
Sprog:Inglês
Udgivet: 2019
Fag:
Article
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC6483838/
https://ncbi.nlm.nih.gov/pubmed/30924938
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/jimd.12094
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