To determine whether whole body protein kinetics are altered in Duchenne mu
scular dystrophy (DMD), six 9 +/- 1-year-old children with DMD and five wei
ght and height matched controls, received intravenous infusion of L-[1-C-13
]leucine and L-[2-N-15]glutamine in the post-absorptive state. Glutamine ra
te of appearance was approximate to 24% lower in DMD boys than in controls
(321 +/- 22 vs 425 +/- 37 mu mol kg(-1) h(-1) P<0.05) resulting from a 32%
decrease in glutamine de novo synthesis (230 +/- 21 vs 340 +/- 34 mu mol kg
(-1) h(-1) P<0.05). Whereas there was no difference between groups in estim
ates of protein degradation and synthesis, leucine oxidation rate was 44% h
igher in DMD boys than in controls (23 +/- 2 vs 16 +/- 2 mu mol kg(-1) h(-1
), P<0.05). The data suggest that the dramatic mucle mass loss observed in
DMD boys is associated with a) significant protein wasting, since increased
leucine oxidation reflects a more negative whole body leucine balance, and
b) a significant decrease in glutamine availability in the postabsorptive
state. Glutamine might therefore be a 'conditionally essential' amino-acid
in DMD. (C) 1999 Harcourt Publishers Ltd.