These Smo/Smo transgenic mice express the constitutively active point mutation, SmoA1, of the mouse smoothened homolog (Drosophila) (Smo) gene, under the control of the 1kb mouse neurogenic differentiation 2 (Neurod2) promoter. Transgene expression is specific to cerebellar granule cells, as observed by His tag staining. Tumor incidence in mice homozygous for the transgene is 85% by one month of age and 94% by two months of age, with an average age of onset of related traits being four months. Traits associated with advanced tumors include enlarged posterior fossa, and/or tilted head, and hunched posture. Weight loss and an ungroomed appearance are also common. The disease progresses to leptomeningeal metastasis of the brain and spine. Once clinical symptoms are noted, survival is one to two weeks. This mutant mouse strain may be useful in studies of medulloblastoma and metastasis.

These transgenic mice express the constitutively active point mutation, SmoA1, of the mouse smoothened homolog (Drosophila) (Smo) gene, under the control of the mouse neurogenic differentiation 2 (Neurod2) promoter, with transgene expression specific to cerebellar granule cells. Homozygous mice show an increased medulloblastoma incidence. This transgenic mouse strain may be useful in studies of medulloblastoma and metastasis.

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