Hereditary renal cell carcinoma in the Eker rat: a unique animal model for the study of cancer susceptibility.

A class of genes, the so-called tumor suppressor genes or anti-oncogenes, was originally identified as being responsible for germ-line transmission of cancer susceptibility in humans. Tumor suppressor genes are recessive at the cellular level with respect to oncogenesis but often manifest as dominantly inherited familial cancer syndromes. This type of cancer syndrome arises in the Eker rat due to a genetic defect in the tuberous sclerosis 2 (Tsc2) gene. The Eker rat familial cancer syndrome serves as a unique animal model in which to study the molecular pathways of renal tubular epithelial carcinogenesis as well as a valuable tool for studies that examine how chemical carcinogens interact with cancer susceptibility genes.