The Nihon rat is a model of renal carcinoma found in a colony of Sprague-Dawley rats in Japan (Okimoto et al. 2004). It contains a single nucleotide insertion in the region of the rat chromosome 10 (an area homologous with human chromosome 17p11.2) producing a frameshift and premature stop codon. In heterozygotes, renal carcinomas develop from early pre-neoplastic lesions, seen as early as three weeks of age, into adenomas by eight weeks of age, with complete penetrance of this renal carcinoma gene by the age of six months. The renal carcinomas that develop in heterozygotes are predominantly clear cell, as is the case in the Ecker rat, a TSC animal model which carries a single gene mutation in the TSC2 gene as the cause of renal carcinoma. The homozygous mutant condition is lethal at an early stage of fetal life in the Nihon rat. LOH at the FLCN locus in ten of eleven primary renal carcinomas is detectable, fitting the Knudson 2-hit model. Okimoto et al, (2004) concluded that the Nihon rat provides insights into a tumour suppressor gene that is related to renal carcinogenesis and an animal model of human BHD syndrome.

Further details: Lab essentials – BHD Animal Models

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