Pheochromocytomas and C-cell thyroid neoplasms in transgenic c-mos mice: a model for the human multiple endocrine neoplasia type 2 syndrome.

摘要: Transgenic mice carrying and expressing a mos protooncogene, linked to the Moloney murine sarcoma virus long terminal repeat, develop severe neurological defects lens abnormalities. Here we report that after latent periods, in three of four these transgenic lines high frequency multicentric pheochromocytomas and/or medullary thyroid neoplasms. The pattern tumor formation is remarkably similar human autosomal dominantly inherited neoplastic syndrome, multiple endocrine neoplasia type 2 (MEN 2), tumors from animals display same neuroendocrine marker staining as seen MEN 2. similarity between pathologies presentation patterns patients suggests they may arise through related pathways. varies line-dependent manner indicating there interaction transgene genetic background. Moreover, when non-tumor-bearing line crossed different mouse background, F1 offspring phenotype. These studies indicate penetrance dominant phenotype dependent on both integration site