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Large neutral amino acids (LNAA) were corrected post transplant (Tx) in intermediate maple syrup urine disease (iMSUD) mouse sera and brains at 100 days. (A, D) branched chain amino acids (BCAA) and alloisoleucine. (B, E) BCAA to alanine ratio. (C, F) selected LNAA. hAEC, human amnion epithelial cell; WT, wildtype; *, p<0.05; **, p<0.01; ***, p<0.001, one-way ANOVA with Tukey post-hoc analysis.
