Figure 2From: Production and characterization of murine models of classic and intermediate maple syrup urine diseaseBiochemical characterization of the classic MSUD murine model. A, BCKDH enzyme activity in liver of newborn wild type control (+/+), heterozygous (+/-), and homozygous (-/-) knockout mice. Enzyme activity was significantly reduced in +/- liver compared to +/+, and was below the level of detection in -/- liver. B, Total BCAA concentrations in blood of mice. Total BCAA represent the sum of leucine, isoleucine, and valine. Total BCAA concentrations in blood from -/- mice were significantly elevated compared to +/+ and +/-. C, Ratio of total BCAA to alanine in blood of mice. This ratio was significantly elevated in -/- mice compared to +/+ and +/- mice. The numbers on the bars indicates the number of mice analyzed. *, Significantly different from +/+ (P < 0.001); **, significantly different from +/+ and +/- (P < 0.001).Back to article page