Through profiling and analyzing large numbers of human tumors, aberrations at multiple levels (gene, transcription, protein, and epigenetic level) can be identified and applied to development of novel treatments.[4–6] Recently, the WHO has introduced molecular parameters, such as mutations in the isocitrate dehydrogenase (IDH) 1 and 2 genes, and 1p/19q co-deletion, to define glioma entities.[7] These updates reflect the consideration of genetic and genomic contributions to biological behaviors of gliomas. Here, IDH3A is linked to central nervous system cancer.