Document Detail


Normal rates of whole-body fat oxidation and gluconeogenesis after overnight fasting and moderate-intensity exercise in patients with medium-chain acyl-CoA dehydrogenase deficiency.
MedLine Citation:
PMID:  22976767     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
BACKGROUND: Impairments in gluconeogenesis have been implicated in the pathophysiology of fasting hypoglycemia in medium-chain acyl-CoA dehydrogenase deficiency. However, whole body glucose and fat metabolism have never been studied in vivo. METHODS: Stable isotope methodology was applied to compare fat and glucose metabolism between four adult patients with MCADD and four matched controls both at rest and during 1.5 h of moderate-intensity exercise. Additionally, intramyocellular lipid and glycogen content and intramyocellular acylcarnitines were assessed in muscle biopsies collected prior to and immediately after cessation of exercise. RESULTS: At rest, plasma FFA turnover was significantly higher in patients with MCADD, whereas the plasma FFA concentrations did not differ between patients and controls. Blood glucose kinetics did not differ between groups both at rest and during exercise. Palmitate and FFA turnover, total fat and carbohydrate oxidation rates, the use of muscle glycogen and muscle derived triglycerides during exercise did not differ between patients and controls. Plasma FFA oxidation rates were significantly lower in patients at the latter stages of exercise. Free carnitine levels in muscle were lower in patients, whereas no differences were detected in muscle acetylcarnitine levels. CONCLUSIONS: Whole-body or skeletal muscle glucose and fat metabolism were not impaired in adult patients with MCADD. This implies that MCADD is not rate limiting for energy production under the conditions studied. In addition, patients with MCADD have a higher FFA turnover rate after overnight fasting, which may stimulate ectopic lipid deposition and, as such, make them more susceptible for developing insulin resistance.
Authors:
Hidde H Huidekoper; Mariëtte T Ackermans; René Koopman; Luc J C van Loon; Hans P Sauerwein; Frits A Wijburg
Related Documents :
16567757 - Prescribing exercise as preventive therapy.
23091517 - Prophylactic effects of swimming exercise on autophagy-induced muscle atrophy in diabet...
2915617 - Health maintenance issues of the elderly. exercise.
10493287 - Exercise counseling by primary care physicians in the era of managed care.
1306187 - Cariogenic changes in dental enamel of boys and girls in relation to salivary propertie...
10843897 - Exercise training restores adenosine-induced relaxation in coronary arteries distal to ...
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-9-14
Journal Detail:
Title:  Journal of inherited metabolic disease     Volume:  -     ISSN:  1573-2665     ISO Abbreviation:  J. Inherit. Metab. Dis.     Publication Date:  2012 Sep 
Date Detail:
Created Date:  2012-9-14     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7910918     Medline TA:  J Inherit Metab Dis     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Department of Pediatrics, University of Amsterdam, Amsterdam, The Netherlands.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


Previous Document:  Dynamic changes of lipid profile in Romanian patients with Gaucher disease type 1 under enzyme repla...
Next Document:  Molecular characterization of 355 mucopolysaccharidosis patients reveals 104 novel mutations.