Document Detail

Hepatic anaplerotic outflow fluxes are redirected from gluconeogenesis to lactate synthesis in patients with Type 1a glycogen storage disease.
MedLine Citation:
PMID:  19558966     Owner:  NLM     Status:  MEDLINE    
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.
John G Jones; Paula Garcia; Cristina Barosa; Teresa C Delgado; Luisa Diogo
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2009-02-04
Journal Detail:
Title:  Metabolic engineering     Volume:  11     ISSN:  1096-7184     ISO Abbreviation:  Metab. Eng.     Publication Date:  2009 May 
Date Detail:
Created Date:  2009-08-12     Completed Date:  2009-12-31     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9815657     Medline TA:  Metab Eng     Country:  Belgium    
Other Details:
Languages:  eng     Pagination:  155-62     Citation Subset:  IM    
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.
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MeSH Terms
Acetyl Coenzyme A / metabolism
Carbon Isotopes / chemistry
Citric Acid Cycle / physiology*
Gluconeogenesis / physiology*
Glucose / metabolism*
Glucose-6-Phosphatase / metabolism
Glutamine / blood
Glycerol / metabolism
Glycogen Storage Disease Type I / metabolism*
Lactates / blood
Liver / metabolism*
Pyruvic Acid / metabolism
Young Adult
Reg. No./Substance:
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

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