To date, IDH mutations only in the isoforms 1 and 2 – localized in cytoplasm and mitochondria, respectively – are the cause of tumors in the central nervous system of glial origin such as astrocytomas, oligodendrogliomas and secondary glioblastomas (55, 56), as well as acute myeloid leukemia [AML (57, 58)] and other solid tumors (For a review, see (59)). Here, IDH1 is linked to acute myeloid leukemia.