Using mice to decipher the molecular genetics of brain tumors

THE PAST DECADE has dramatically increased our knowledge of genetic and molecular alterations in human central nervous system tumors. Important as these alterations are for the molecular classification of tumors their actual roles in tumorigenesis and tumor progression have long remained obscure. Lately, several mouse brain tumor models have been developed that use different gene modification strategies to replicate mutations seen in the human counterpart. These genetic models will allow discrimination between mutations that are causally related to tumor formation and mutations that are a result of tumor progression. These models also provide histologicallly and genetically accurate models for preclinical testing and will perhaps help us identify novel targets for therapies aimed at the mechanistic cause of the disease. We present here a review of current models, with a focus on gliomas and medulloblastomas.