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Atlas of Genetics and Cytogenetics in Oncology and Haematology OPEN ACCESS JOURNAL AT INIST-CNRS Gene Section Short Communication OLIG2 (oligodendrocyte factor 2) lineage transcription David Rowitch Department of Pediatric Oncology, Dana-Farber Cancer Institute, Dana 640D, 44 Binney Street, Boston, MA 02115, USA (DR) Published in Atlas Database: May 2004 Online updated version : http://AtlasGeneticsOncology.org/Genes/OLIG2ID236.html DOI: 10.4267/2042/38095 This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 2.0 France Licence. © 2004 Atlas of Genetics and Cytogenetics in Oncology and Haematology Identity Implicated in Other names: RK17; Olg-2; Bhlhb1; RACK17; HGNC:13226; PRKCBP2; BHLHB1 HGNC (Hugo): OLIG2 Location: 21q22.11 T-cell lymphoma Cytogenetics Translocation t(14;21)(q11.2;q22). Hybrid/Mutated gene T-cell receptor promoter translocated upstream of OLIG2. Abnormal protein None. Brain cancer 329 amino acids; 32 kDa; contains 1 basic helix-loophelix DNA-binding domain (bHLH). Disease Oligodendroglioma, astrocytoma, glioblastoma. Prognosis Variable. Cytogenetics No known genetic abnormalities in OLIG2. Hybrid/Mutated gene n/a Oncogenesis OLIG expression in brain cancer raises possibilities that it could regulate behavior of tumor cell populations but this has not been demonstrated. Expression References Probe(s) - Courtesy Mariano Rocchi, Resources for Molecular Cytogenetics. DNA/RNA Note OLIG2 is an essential transcriptional regulator in motoneuron and oligodendrocyte development. Protein Description Olig2 is expressed in the ventral thalamus of developing mice. Wang J, Jani-Sait SN, Escalon EA, Carroll AJ, de Jong PJ, Kirsch IR, Aplan PD. The t(14;21)(q11.2;q22) chromosomal translocation associated with T-cell acute lymphoblastic leukemia activates the BHLHB1 gene. Proc Natl Acad Sci U S A. 2000 Mar 28;97(7):3497-502 Function Required for oligodendrocyte and motor neuron specification in the spinal cord. Atlas Genet Cytogenet Oncol Haematol. 2004; 8(3) 210 OLIG2 Rowitch D Lu QR, Park JK, Noll E, Chan JA, Alberta J, Yuk D, Alzamora MG, Louis DN, Stiles CD, Rowitch DH, Black PM. Oligodendrocyte lineage genes (OLIG) as molecular markers for human glial brain tumors. Proc Natl Acad Sci U S A. 2001 Sep 11;98(19):10851-6 Hoang-Xuan K, Aguirre-Cruz L, Mokhtari K, Marie Y, Sanson M. OLIG-1 and 2 gene expression and oligodendroglial tumours. Neuropathol Appl Neurobiol. 2002 Apr;28(2):89-94 Azzarelli B, Miravalle L, Vidal R. Immunolocalization of the oligodendrocyte transcription factor 1 (Olig1) in brain tumors. J Neuropathol Exp Neurol. 2004 Feb;63(2):170-9 Marie Y, Sanson M, Mokhtari K, Leuraud P, Kujas M, Delattre JY, Poirier J, Zalc B, Hoang-Xuan K. OLIG2 as a specific marker of oligodendroglial tumour cells. Lancet. 2001 Jul 28;358(9278):298-300 Atlas Genet Cytogenet Oncol Haematol. 2004; 8(3) This article should be referenced as such: Rowitch D. OLIG2 (oligodendrocyte lineage transcription factor 2). Atlas Genet Cytogenet Oncol Haematol. 2004; 8(3):210211. 211