ENU mutagenesis identifies mice with mitochondrial branched-chain aminotransferase deficiency resembling human maple syrup urine disease
J. Clin. Invest. Jer-Yuarn Wu, et al. 113:434 doi:10.1172/JCI19574 [Go to this article.]

Figure 4
Body weight and amino acid changes with diet restricted in BCAAs. (a) Body weight changes of five mice in two litters: affected males (filled squares and filled triangles); affected female (filled circles); unaffected siblings (male, open triangles; female, open circles). Black arrows indicate when the restricted diet (MSUD diet/regular diet, 2:1) was initiated. Open arrows denote that diet was further changed to MSUD diet/regular diet, 3:1. One mouse (filled triangle) died accidentally at day 89. (b) Changes in amino acid concentrations. Light gray bars, affected mice with regular diet; dark gray bars, MSUD diet/regular diet, 2:1; black bars, MSUD diet/regular diet, 3:1; white bars, control ENU mice with regular diet.