Figure 6: FLC is characterized by uniporter-mediated suppression of BCAA catabolism.

(A) Analysis of publicly available gene expression datasets of FLC tumor and non-tumor liver (NTL) show decreased in BCAA catabolism gene expression in FLC (B) Immunoblots of select BCAA catabolism enzymes from paired non-tumor (N) and FLC tumor (T) lysates and pooled quantification of protein levels normalized to TOM20 levels; statistical significance was determined by one-sample t-test. (C) Immunoblots and quantification of select BCAA catabolism proteins and uniporter components in AML12 cells; statistical significance was determined by one-sample t-test; n=4. (D) MCU knockdown increases expression of BCAA catabolism pathway proteins in FLC clones; immunoblots of select pathway proteins and uniporter components are shown. All error bars indicate standard deviation; * indicates a p-value < 0.05, ** indicates a p-value < 0.01, and *** indicates a p-value < 0.001.