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)