p27 is a haploinsufficient tumor suppressor in the MENX multiple endocrine neoplasia syndrome in rats

As overwhelming evidence coming from transgenic mouse models but also from MEN4 patients seem to suggest that loss or inactivation of a single p27 allele plays an important role in neuroendocrine tumorigenesis, we decided to perform a detailed analysis of the phenotype of rats heterozygous for the MENX-associated germline Cdkn1b mutation. We here show that the reduction to a single functional p27 allele predisposes MENX heterozygous rats to the development of neuroendocrine malignancies.

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Molatore S, Kügler A, Irmler M, Wiedemann T, Neff F, Feuchtinger A, Beckers J, Robledo M, Roncaroli F, Pellegata NS