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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).