Table 1.

Metabolic changes in brains of iMSUD mice following hAEC transplant.

	iMSUD vs. WT	iMSUD-hAEC vs. WT	iMSUD-hAEC vs. iMSUD
Leucine	↑ >40-fold	=	↓ >60%
Isoleucine	↑ >40-fold	↑ 15-fold	↓ >60%
Valine	↑ >20-fold	=	↓ >60%
Alloisoleucine	↑ >120 fold	=	↓ 80%
BCAA/alanine	↑ >10-fold	=	↓ >50%
Phenylalanine	↑ >3-fold	=	↓ >55%
Tyrosine	↑ >5-fold	=	↓ >70%
Methionine	↑ 2.75-fold	=	↓ >35%, NS

An equals sign (=) indicates either a correction or partial correction (see criteria in Methods section). BCAA, branched chain amino acids (leucine, isoleucine, and valine); iMSUD, intermediate maple syrup urine disease; hAEC, human epithelial cell; WT, wildtype.