Words similar to medulloblastomas
Example sentences for: medulloblastomas
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Mutations in the Hedgehog pathway members PTCH , PTCH 2 , SUFU and Smo , have all been identified in sporadic medulloblastomas as well, with approximately 25% of cases containing mutations affecting these genes [ 5 6 7 8 9 ] . A murine medulloblastoma model was recently developed by disruption of the PTCH gene, with medulloblastoma-like tumors arising in 10-15% of heterozygotes by 9 months of age [ 10 11 ] . Breeding PTCH mice to p53 knockout animals markedly increased tumor incidence.
We have previously shown that nuclear β-catenin is present in 18% of sporadic medulloblastomas [ 18 ] .
In order to determine if Wnt signaling was aberrantly active in the cerebellar neuroblasts thought to give rise to some medulloblastomas, β-catenin immunostains were performed on newborn Mut-1 and Mut-2 animals.
Mice heterozygous for PTCH and lacking p53 all developed medulloblastomas by 3 months of age [ 12 ] .
In man, three inherited syndromes associated with medulloblastomas have been described: Turcot's, Gorlin's and Li Fraumeni (reviewed in [ 1 ] ). Li Fraumeni syndrome is caused by inherited mutations in the p53 tumor suppressor gene.