Medulloblastoma is the most common malignant pediatric brain tumor. Despite its prevalence and importance in pediatric neuro-oncology, the genes and pathways responsible for its initiation, maintenance, and progression remain poorly understood. Genetically engineered mouse models are an essential tool for uncovering the molecular and cellular basis of human diseases, including cancer, and serve a valuable role as preclinical models for testing targeted therapies. In this review, we summarize how such models have been successfully applied to the study of medulloblastoma over the past decade and what we might expect in the coming years.
Animals ; Cerebellar Neoplasms ; genetics ; metabolism ; pathology ; Disease Models, Animal ; Genetic Engineering ; Hedgehog Proteins ; metabolism ; Humans ; Medulloblastoma ; genetics ; metabolism ; pathology ; Mice ; Mice, Transgenic ; Mutation ; Patched Receptors ; RNA Interference ; RNA, Small Interfering ; genetics ; Receptors, Cell Surface ; genetics ; metabolism ; Receptors, G-Protein-Coupled ; metabolism ; Signal Transduction ; Smoothened Receptor ; Tumor Suppressor Protein p53 ; genetics ; metabolism