Extended Data Fig. 7: Ectopic BCAT1 Expression Ameliorates BCAT2 Deficiency-Induces Metabolic Defects.
From: Metabolon formation regulates branched-chain amino acid oxidation and homeostasis

(a) Western blot analysis to validate the cytosolic expression of BCAT1 in WT and Bcat1OE kidney lysates. (b) Western blot results are shown for the validation of Bcat1OE and Bcat1OE;Bcat2−/− mice and the expression of enzymes in the BCAA metabolic pathway in major tissues. (c) Fractional enrichment of KIV_M + 5 in the plasma of the indicated genotypes during the 2-hour stable isotope infusion experiments. Results are mean ± SD (N = 4 WT, 4 Bcat2−/−, 3 Bcat1OE and 3 Bcat1OE;Bcat2−/− mice). (d) Relative abundance of Val_M + 0,5 and KIV_M + 0,5 in plasma samples before (0 hour) and after (2 hour) KIV_M + 5 stable isotope infusion with the number (N) of independent samples shown. Results are mean ± SD and analysed by two-way ANOVA. (e) Glutamate/α-KG ratios in major tissues of the indicated genotypes with the number (N) of independent samples shown. Results are mean ± SD and analysed by one-way ANOVA. (f) NAD+/NADH ratio in major organs of the indicated genotypes with the number (N) of samples shown. Results are mean ± SD and analysed by two-way ANOVA. (g) Insulin levels in the serum of the indicated genotypes. Results are mean ± SD (N = 5 WT, 4 Bcat2−/−, 4 Bcat1OE and 4 Bcat1OE;Bcat2−/− mice) and analysed by one-way ANOVA.