Blunted fat oxidation upon submaximal exercise is partially compensated by enhanced glucose metabolism in children, adolescents, and young adults with Barth syndrome.
Barth syndrome
exercise
fatty acid
mitochondria
Journal
Journal of inherited metabolic disease
ISSN: 1573-2665
Titre abrégé: J Inherit Metab Dis
Pays: United States
ID NLM: 7910918
Informations de publication
Date de publication:
05 2019
05 2019
Historique:
received:
06
11
2018
accepted:
27
03
2019
pubmed:
30
3
2019
medline:
26
6
2020
entrez:
30
3
2019
Statut:
ppublish
Résumé
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 fat metabolism during rest, submaximal exercise, and postexercise rest in children, adolescents, and young adults with BTHS and unaffected controls and examine their relationships with cardioskeletal energetics and function. Children/adolescents and young adults with BTHS (n = 29) and children/adolescent and young adult control participants (n = 28, total n = 57) underwent an infusion of 6'6'H2 glucose and U-
Identifiants
pubmed: 30924938
doi: 10.1002/jimd.12094
pmc: PMC6483838
mid: NIHMS1021443
doi:
Substances chimiques
Blood Glucose
0
Fatty Acids
0
Banques de données
ClinicalTrials.gov
['NCT01625663']
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Langues
eng
Sous-ensembles de citation
IM
Pagination
480-493Subventions
Organisme : NHLBI NIH HHS
ID : R01 HL107406
Pays : United States
Organisme : NIDDK NIH HHS
ID : P30 DK056341
Pays : United States
Organisme : NICHD NIH HHS
ID : T32 HD007434
Pays : United States
Organisme : NCATS NIH HHS
ID : UL1 TR000448
Pays : United States
Organisme : NIDDK NIH HHS
ID : P30 DK020579
Pays : United States
Organisme : NCATS NIH HHS
ID : UL1 TR002345
Pays : United States
Organisme : NIBIB NIH HHS
ID : K01 EB010171
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL136759
Pays : United States
Informations de copyright
© 2019 SSIEM.
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