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)