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Hepatic anaplerotic outflow fluxes are redirected from gluconeogenesis to lactate synthesis in patients with Type 1a glycogen storage disease

Paper ID Volume ID Publish Year Pages File Format Full-Text
31913 44852 2009 8 PDF Available
Title
Hepatic anaplerotic outflow fluxes are redirected from gluconeogenesis to lactate synthesis in patients with Type 1a glycogen storage disease
Abstract

Hepatic glucose production and relative Krebs cycle fluxes (indexed to a citrate synthase flux of 1.0) were evaluated with [U-13C]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 13C-isotopomers were analyzed by 13C 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 13C-isotopomers derived from the Krebs cycle suggests that lactate was the main anaplerotic product in GSD1a patients.

Keywords
13C NMR; Pyruvate recycling; Anaplerosis; Gluconeogenesis; Isotopomer analysis
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Hepatic anaplerotic outflow fluxes are redirected from gluconeogenesis to lactate synthesis in patients with Type 1a glycogen storage disease
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Publisher
Database: Elsevier - ScienceDirect
Journal: Metabolic Engineering - Volume 11, Issue 3, May 2009, Pages 155–162
Authors
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Subjects
Physical Sciences and Engineering Chemical Engineering Bioengineering
Get Full-Text Now
Don't Miss Today's Special Offer
Price was $35.95
You save - $31
Price after discount Only $4.95
100% Money Back Guarantee
Full-text PDF Download
Online Support
Any Questions? feel free to contact us