Figure 4

BHDS-derived tumors possess characteristics of an active PGC-1α-TFAM signaling axis. A) Schematic of FLCN interacting proteins in signal transduction pathway. B) Relative gene expression levels for tuberous sclerosis 1 (TSC1) and folliculin interacting protein 2 (FNIP2) proteins in tumors from patients with BHDS, ON, CH, and the other RCC subtypes from Figure 1A. C) An independent PGC-1α signature from over-expression of PGC-1α in HepG2 cells (GSE5968), showing the top 150 genes (rows) that are up-regulated in PGC-1α over-expressing cells compared to controls (columns). Red indicates high expression and blue indicates low expression. D) Correlation of empirically-derived PGC-1α signature represented in C compared to the PGC signature from Figures 3A and 3D, applied to the six RCC subtypes, using Pearson's correlation. E) Relative expression of the TFAM transcription factor involved in mitochondrial biogenesis (all p ≤ 0.01) in gene expression array data from BHD, CH, ON, and the remaining RCC subtypes, as well as non-diseased tissue and from qRT-PCR validation of a subset of those samples.