Over the last decade isocitrate dehydrogenase (IDH) mutation, chromosome 1p/19q deletion, MGMT promoter methylation, TERT promoter mutation and histone mutation have been identified as biomarkers and play a central role for classification of gliomas and treatment decisions [10–12]. However, the molecular understanding of gliomas is still limited, the treatment of gliomas is full of challenges and prognosis is not optimistic. Here, IDH3A is linked to glioma.