title: New Molecular Considerations for Glioma: IDH, ATRX, BRAF, TERT, H3 K27M.
abstract: This review will discuss the role of several key players in glioma classification and biology, namely isocitrate dehydrogenase 1 and 2 (IDH1/2), alpha thalassemia/mental retardation syndrome X-linked (ATRX), B-Raf (BRAF), telomerase reverse transcriptase (TERT), and H3K27M.
  
  
  
  IDH1/2 mutation delineates oligoden-droglioma, astrocytoma, and secondary glioblastoma (GBM) from primary GBM and lower-grade gliomas with biology similar to GBM. Additional mutations including TERT, 1p/19q, and ATRX further guide glioma classification and diagnosis, as well as pointing directions toward individualized treatments for these distinct molecular subtypes. ATRX and TERT mutations suggest the importance of telomere maintenance in gliomagenesis. BRAF alterations are key in certain low-grade gliomas and pediatric gliomas but rarely in high-grade gliomas in adults. Histone mutations (e.g., H3K27M) and their effect on chromatin modulation are novel mechanisms of cancer generation and uniquely seen in midline gliomas in children and young adults. Over the past decade, a remarkable accumulation of knowledge from the genomic study of gliomas has led to reclassification of tumors, new understanding of oncogenic mechanisms, and novel treatment strategies.
authors.affiliation: Department of Neurosurgery, Clinical Neurosciences Center, University of Utah, Salt Lake City, UT, USA.
authors.forename: Michael
authors.initials: M
authors.lastname: Karsy
authors.affiliation: Department of Neurosurgery, Clinical Neurosciences Center, University of Utah, Salt Lake City, UT, USA.
authors.forename: Jian
authors.initials: J
authors.lastname: Guan
authors.affiliation: Huntsman Cancer Institute, University of Utah, Salt Lake City, UT, USA.|Department of Internal Medicine, University of Utah, Salt Lake City, UT, USA.
authors.forename: Adam L
authors.initials: AL
authors.lastname: Cohen
authors.affiliation: Department of Neurosurgery, Clinical Neurosciences Center, University of Utah, Salt Lake City, UT, USA.|Huntsman Cancer Institute, University of Utah, Salt Lake City, UT, USA.
authors.forename: Randy L
authors.initials: RL
authors.lastname: Jensen
authors.affiliation: Department of Neurosurgery, Clinical Neurosciences Center, University of Utah, Salt Lake City, UT, USA. howard.colman@hci.utah.edu.|Huntsman Cancer Institute, University of Utah, Salt Lake City, UT, USA. howard.colman@hci.utah.edu.|Department of Neurosurgery, The University of Utah, 175 North Medical Dr. East, Salt Lake City, UT, 84132, USA. howard.colman@hci.utah.edu.
authors.forename: Howard
authors.initials: H
authors.lastname: Colman
mesh_terms: D001932:Brain Neoplasms / Q000235:genetics; D004265:DNA Helicases / Q000235:genetics*; D023281:Genomics; D005910:Glioma / Q000145:classification* / Q000175:diagnosis / Q000235:genetics*; D006657:Histones / Q000235:genetics*; D006801:Humans; D007521:Isocitrate Dehydrogenase / Q000235:genetics*; D009154:Mutation; D009687:Nuclear Proteins / Q000235:genetics*; D048493:Proto-Oncogene Proteins B-raf / Q000235:genetics*; D019098:Telomerase / Q000235:genetics*; D000075924:X-linked Nuclear Protein
chemical_list: D006657:Histones; D009687:Nuclear Proteins; D007521:Isocitrate Dehydrogenase; C482119:BRAF protein, human; D048493:Proto-Oncogene Proteins B-raf; C509186:TERT protein, human; D019098:Telomerase; D004265:DNA Helicases; C092332:ATRX protein, human; D000075924:X-linked Nuclear Protein
keywords: ATRX; BRAF; Glioma; H3K27M; IDH; TERT