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Table 2 Summary of additional blood amino acid levels in the intermediate MSUD model and littermate controls as determined by tandem mass spectrometry. Samples were collected from mice that were 4–6 weeks of age. 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)
control 225.2 ± 36.6 (11) 106.5 ± 10.5 (11) 67.8 ± 7.6 (11)
525A 175.0 ± 28.7 (10) 68.4 ± 7.7** (10) 58.0 ± 7.0 (10)
A 144.1 ± 16.9* (10) 65.9 ± 8.9** (10) 37.7 ± 6.5**,*** (10)
  1. *: Significantly different from control (P < 0.05).
  2. **: Significantly different from control (P < 0.01).
  3. ***: Significantly different from Line 525A (P < 0.05).