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FLASH GENE
Symbol OLIG2 contributors: shn - updated : 29-08-2014
HGNC name oligodendrocyte lineage transcription factor 2
HGNC id 9398
Location 21q22.11      Physical location : 34.398.238 - 34.401.500
Synonym name
  • basic domain, helix-loop-helix protein, class B, 1
  • class B basic helix-loop-helix protein 1
  • class E basic helix-loop-helix protein 19
  • human protein kinase C-binding protein RACK17
  • oligodendrocyte transcription factor 2
  • oligodendrocyte-specific bHLH transcription factor 2
  • protein kinase C binding protein 2
  • protein kinase C-binding protein 2
  • protein kinase C-binding protein RACK17
    • Synonym symbol(s) BHLHB1, RACK17, PRKCBP2, OLIGO2, bHLHe19
    DNA
    TYPE functioning gene
    STRUCTURE 3.26 kb     2 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status confirmed
    Map cen - D21S261 - D21S262 - OLIG2 - D21S1898 - D21S1910 - qter
    Physical map
    C21orf45 21q22.11 chromosome 21 open reading frame 45 C21orf61 21q22.1 chromosome 21 open reading frame 61 C21orf119 21 chromosome 21 open reading frame 119 C21orf63 21q22.11 chromosome 21 open reading frame 63 C21orf77 21q22.11 chromosome 21 open reading frame 7 TCP10L 21q22.11 t-complex 10 (mouse)-like C21orf59 21q22.1 chromosome 21 open reading frame 59 LOC391279 21 similar to putative taste receptor HTR2 SYNJ1 21q22.1-q22.2 synaptojanin 1 C21orf66 21q22.11 chromosome 21 open reading frame 66 C21orf62 21q22.1 chromosome 21 open reading frame 62 OLIG2 21q22.11 oligodendrocyte lineage transcription factor 2 C21orf54 21q22.11 chromosome 21 open reading frame 54 IFNAR2 21q22.1 interferon (alpha, beta and omega) receptor 2 IL10RB 21q22.1-q22.2 interleukin 10 receptor, beta IFNAR1 21q22.1 interferon (alpha, beta and omega) receptor 1 IFNGR2 21q22.1 interferon gamma receptor 2 (interferon gamma transducer 1) C21orf4 21q22.1-q22.2 chromosome 21 open reading frame 4 RPS5L 21q22.11 ribosomal protein S5-like C21orf55 21q22.11 chromosome 21 open reading frame 55 GART 21q22.1 phosphoribosylglycinamide formyltransferase, phosphoribosylglycinamide synthetase, phosphoribosylaminoimidazole synthetase SON 21q22.1-q22.2 SON DNA binding protein DONSON 21q22.1 downstream neighbor of SON CRYZL1 21q22.1 crystallin, zeta (quinone reductase)-like 1 ITSN1 21q22.1-q22.2 intersectin 1 (SH3 domain protein)
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    2 - 2505 - 323 oligodendroglial tumors of the brain, pMN domain of the spinal cord - 11955447
    EXPRESSION
    Type restricted
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Nervousbrain     Homo sapiens
     spinal cord   specific
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Nervouscentral    Homo sapiens
    cells
    SystemCellPubmedSpeciesStageRna symbol
    Nervousneuroectodermal cell Homo sapiens
    Nervousoligodendrocyte Homo sapiens
    cell lineage gliogenic progenitors in the postnatal subventricular zone
    cell lines embryonic and adult NG2 cells
    fluid/secretion
    at STAGE
    physiological period embryo
    Text developing astrocytes
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • in the N-terminus a triple serine motif phosphorylated in cycling neural progenitors but not in their differentiated progeny
  • helix-loop-helix (HLH) DNA binding domain
    • conjugated
    HOMOLOGY
    interspecies ortholog to Olig 2, Rattus norvegicus
    ortholog to Olig 2, Mus musculus
    ortholog to olig2, danio rerio
    ortholog to OLIG2, Pan troglodytes
    Homologene
    FAMILY
  • basic helix loop helix (BHLH) family of transcription family
    • CATEGORY transcription factor
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm
    intracellular,nucleus
    basic FUNCTION
  • plays in cooperation with Neurogenin2 vital roles in the coordinated induction of pan-neuronal and subtype-specific properties of motoneurons
  • required for oligodendrocytes and motor neuron specification in the spinal cord
  • controlling the development of neural crest cells, astrocytes and oligodendrocytes, three neuroectodermal-derived cells
  • plays critical roles in oligodendrocyte and motor neuron development
  • export of OLIG2, stimulated AKT, by from the nucleus of neural stem cell is essential for the astrocyte differentiation
  • repressor of neurogenesis in cells reacting to brain injury and open innovative perspectives toward evoking endogenous neuronal repair
  • having repressor function, both sufficient and necessary to prevent neuronal differentiation and to direct subventricular zone progenitors toward astrocytic and oligodendrocytic fates
  • required for proliferation of neural progenitors and for glioma formation
  • playing a crucial role in white matter astrocyte development, but not in the cortical gray matter astrocytes
  • role in gliogenesis in the dorsal pallium
  • play a role in ventral telencephalic neurogenesis
  • required for oligodendrocyte specification and differentiation
  • functions as a prepatterning factor to direct SMARCA4 to oligodendrocyte-specific enhancers
  • basic helix-loop-helix transcription factor necessary for oligodendroglial development and expressed continuously throughout the lineage
  • is involved in correct formation of the prethalamus, which leads to exclusion of the EPHA3-expressing region and is crucial for proper thalamocortical axons (TCAs) formation
    • CELLULAR PROCESS nucleotide, transcription
    PHYSIOLOGICAL PROCESS development , nervous system , neurogenesis
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • NK2 homeobox 2, Nkx2.2
  • SRY (sex determining region Y)-box 10, SOX10 and SOXE
  • NEUROG2 to modulate gene expression in motor neuron progenitor cells
  • ID2 (inhibited posttranslationally through the interaction with ID2)
  • SMARCA4 is an integral component of the transcriptional control of myelination via interaction with OLIG2 at the onset of oligodendrocyte progenitor cells differentiation
  • stage-specific regulatory role for OLIG2, mediated by OLIG1 that conveys opposing functions on the differentiation and maturation of oligodendrocytes
  • LGR5 is regulated by OLIG2, which is important for both neurogenesis and stem-like cells in glioblastoma (GSCs) maintenance
  • PROX1 is a transcription repressor and downstream target of proneural genes, that suppresses OLIG2 expression and therefore controls ventral spinal cord patterning
    • cell & other
    REGULATION
    Other phosphorylation regulates OLIG2 cofactor choice and the motor neuron-oligodendrocyte fate switch
    proliferative function of OLIG2 is controlled by developmentally regulated phosphorylation of a conserved triple serine motif within the N-terminal domain
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral       gain of function
    activated in T cell lymphoblastic leukemia with t(14;21) (q11.2;q22)
    constitutional     --over  
    acute and chronic brain injury
    tumoral     --low  
    may be related to the malignant behavior of human glioblastoma
    tumoral     --over  
    in oligodendroglial tumors
    constitutional   amplification    
    Olig1 and Olig2 triplication causes developmental brain defects in Down syndrome
    constitutional     --over  
    over-expression inhibits neural progenitor proliferation through changes in potassium channel activity, thereby contributing to the reduced neuronal numbers and brain size in Down syndrome
    Susceptibility to schizophrenia
    Variant & Polymorphism SNP SNP rs762178 in OLIG2 seems to be a potential candidate in altering risk for schizophrenia in the Chinese Han population
    Candidate gene
    Marker
  • a molecular marker for human glial brain tumors
  • a marker for the diagnosis of oligodendroglial tumours
    • Therapy target
    ANIMAL & CELL MODELS
  • in Olig1(-/-)2(-/-) double-mutant mice motoneurons are largely eliminated, and oligodendrocyte differentiation is abolished
  • mice lacking Olig2 function demonstrates a failure of NG2 cell development at embryonic and perinatal stages
  • in Olig2 deficient mouse, astrocyte development was retarded in the dorsal neocortex, but not in the basal forebrain
  • Olig2 is triplicated and overexpressed in the Ts65Dn mouse (mouse models of Down syndrome) forebrain displaying cognitive deficits