Renal carcinoma development is correlated with FLCN inactivation caused by naturally occurring germline mutations in human BHD patients, the Nihon rat model (C insertion in exon 3 of rat Flcn), German Shepherd dogs resulting in renal cystadenocarcinoma and nodular dermatofibrosis (RCND; H255R mutation in canine Flcn) and by genetically manipulated deletions in mice [21], [24]–[30]. The gene discussed is FLCN; the disease is Birt-Hogg-Dube syndrome.