In studies that restricted their analysis to established RCC predisposition genes (BAP1, EPCAM, FH, FLCN, MET, MITF, MLH1, MSH2, MSH6, PMS2, PTEN, SDHA, SDHB, SDHC, SDHD, TP53, TSC1, TSC2, and VHL), PLP variants were identified in 1–6% of individuals tested [4,5,6,8,11,12]. Here, SDHD is linked to renal cell carcinoma.