Abstract
We determined whether glutamine has a protein anabolic effect in six 8-13-y-old boys with Duchenne muscular dystrophy. Children received a 5-h i.v. infusion of L-[1-13C]leucine and L-[2-15N]glutamine in the postabsorptive state on two consecutive days while drinking: 1) flavored water on one day, and 2) the same drink mixed with L-glutamine (800 μmol·kg-1·h-1), the other day. Oral glutamine administration was associated with an 8% decrease in leucine release from protein breakdown, from 116 ± 5 to 107 ± 6μmol·kg-1h-1 (p < 0.01), and a 35% decrease in leucine oxidation rate from 23 ± 2 to 15 ± 2μmol·kg-1·-1 (p < 0.01), resulting in no change in the nonoxidative leucine disposal, an index of protein synthesis. Whole body glutamine exchange in plasma doubled from 321 ± 22 to 623 ± 24 μmol·kg-1·h-1,p < 0.01, but glutamine from protein degradation and glutamine de novo synthesis both decreased (91 ± 4 versus 84± 5 μmol·kg-1·-1, p < 0.01, and 230 ± 21 versus 163 ± 25μmol·kg-1·h-1, p = 0.02, respectively). These data suggest that acute oral glutamine administration might have a protein-sparing effect in children with Duchenne muscular dystrophy, decreasing estimates of whole body protein degradation and glutamine de novo synthesis, therefore sparing nitrogen precursors.
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Abbreviations
- DMD:
-
Duchenne muscular dystrophy
- KIC:
-
α-ketoisocaproic acid
- E p ,KIC :
-
plasma enrichment of KIC
- E p ,gln :
-
plasma enrichment of glutamine
- E13CO2:
-
13 CO2 enrichment in expired air
- R a ,leu :
-
leucine appearance rate in plasma
- Oxleu:
-
leucine oxidation rate
- VCO2:
-
CO2 production rate
- NOLD:
-
nonoxidative leucine disposal rate
- R a ,gln :
-
whole body glutamine exchange in plasma
- Endo Ra,gln:
-
endogenous glutamine appearance rate in plasma
- B gln :
-
glutamine arising from protein breakdown
- D gln :
-
glutamine de novo synthesis
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Acknowledgements
We acknowledge the superb technical assistance of Brenda K Sager, B.S., Astride Altomare, B.S., M.S., Linda Everline, Debra D'Amore, B.S., M.S., W. Reed Parsons, B.S., and Ed Jones, B.S. We are grateful to Pennye Arehart, R.N., B.S.N., Bernice Rutledge, R.N., Elmyra Morris, R.N., and the Nursing Staff of the Wolfson Children's Hospital for their help in the performance of these studies.
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Supported in part by a grant from the Nemours Foundation and NIH Grant R01/DK51477.
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Hankard, R., Hammond, D., Haymond, M. et al. Oral Glutamine Slows Down Whole Body Protein Breakdown in Duchenne Muscular Dystrophy. Pediatr Res 43, 222–226 (1998). https://doi.org/10.1203/00006450-199802000-00011
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DOI: https://doi.org/10.1203/00006450-199802000-00011
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