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]. The gene discussed is VHL; the disease is renal cell carcinoma.