| Hepatic anaplerotic outflow fluxes are redirected from gluconeogenesis to lactate synthesis in patients with Type 1a glycogen storage disease. | |
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MedLine Citation:
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PMID: 19558966 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Hepatic glucose production and relative Krebs cycle fluxes (indexed to a citrate synthase flux of 1.0) were evaluated with [U-(13)C]glycerol tracer in 5 fed healthy controls and 5 Type 1a glycogen storage disease (GSD1a) patients. Plasma glucose, hepatic glucose-6-phosphate (G6P) and glutamine (13)C-isotopomers were analyzed by (13)C NMR via blood sampling and chemical biopsy. In healthy subjects, 35+/-14% of plasma glucose originated from hepatic G6P while GSD1a patients had no detectable G6P contribution. Compared to controls, GSD1a patients had an increased fraction of acetyl-CoA from pyruvate (0.5+/-0.2 vs. 0.3+/-0.1, p<0.01), and increased pyruvate recycling fluxes (14.4+/-3.8 vs. 8.7+/-2.8, p<0.05). Despite negligible gluconeogenic flux, net anaplerotic outflow was not significantly different from controls (2.2+/-0.8 vs. 1.3+/-0.5). The enrichment of lactate with (13)C-isotopomers derived from the Krebs cycle suggests that lactate was the main anaplerotic product in GSD1a patients. |
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Authors:
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John G Jones; Paula Garcia; Cristina Barosa; Teresa C Delgado; Luisa Diogo |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2009-02-04 |
Journal Detail:
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Title: Metabolic engineering Volume: 11 ISSN: 1096-7184 ISO Abbreviation: Metab. Eng. Publication Date: 2009 May |
Date Detail:
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Created Date: 2009-08-12 Completed Date: 2009-12-31 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9815657 Medline TA: Metab Eng Country: Belgium |
Other Details:
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Languages: eng Pagination: 155-62 Citation Subset: IM |
Affiliation:
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NMR Research Unit, Department of Biochemistry and Center for Neurosciences and Cell Biology, Faculty of Sciences and Technology, University of Coimbra, 3001-401 Coimbra, Portugal. john.jones@netc.pt |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Acetyl Coenzyme A
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metabolism Adolescent Carbon Isotopes / chemistry Child Citric Acid Cycle / physiology* Female Gluconeogenesis / physiology* Glucose / metabolism* Glucose-6-Phosphatase / metabolism Glutamine / blood Glycerol / metabolism Glycogen Storage Disease Type I / metabolism* Humans Lactates / blood Liver / metabolism* Male Pyruvic Acid / metabolism Young Adult |
| Chemical | |
Reg. No./Substance:
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0/Carbon Isotopes; 0/Lactates; 127-17-3/Pyruvic Acid; 50-99-7/Glucose; 56-81-5/Glycerol; 56-85-9/Glutamine; 72-89-9/Acetyl Coenzyme A; EC 3.1.3.9/Glucose-6-Phosphatase |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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