Abstract
Isocitrate dehydrogenase (IDH) mutations are biomarkers to classify diffuse gliomas into biologically similar subgroups. Tremendous efforts have been made to understand the biology of IDH-mutant gliomas at the genetic, epigenetic, transcriptional, and protein levels. Preclinical models that recapitulate human tumor biology are crucial not only to our understanding of IDH mutations in gliomagenesis, but also in testing of novel therapeutic agents that may lead to more effective therapies for IDH-mutant glioma patients.