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Table 1 Summary of additional blood amino acid levels in the classic MSUD model and control littermates as determined by tandem mass spectrometry. All samples were collected on the day of birth. All values are mean +/- SEM.

From: Production and characterization of murine models of classic and intermediate maple syrup urine disease

MSUD Genotype Alanine Glutamate Glutamine
(μmole/L)
+/+ 196.1 ± 23.1 (6) 229.0 ± 12.0 (6) 76.8 ± 3.0 (6)
+/- 167.6 ± 9.9 (13) 251.0 ± 12.8 (13) 72.3 ± 1.6 (13)
-/- 94.6 ± 11.6* (5) 107.8 ± 4.8* (5) 50.4 ± 2.9* (5)
  1. *: Significantly different from +/+ and +/- (P < 0.001).