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GATA2 (GATA binding protein 2)

Identity

Other namesMGC2306
NFE1B
HGNC (Hugo) GATA2
LocusID (NCBI) 2624
Location 3q21.3
Location_base_pair Starts at 128198265 and ends at 128207373 bp from pter ( according to hg19-Feb_2009)  [Mapping]

DNA/RNA

Description Genomic DNA 13,759bp, 6 exons (one non coding).
Transcription mRNA 3383bp, trancription is oriented from telomere to centromere. It contains 2 alternative first exons, one distal (IS) specifically transcribed in hematopoietic and neural cells, and a proximal one (IG) transcribed in other cell types.

Protein

Description Contains 2 zinc finger domains, ZF1 (aa294 to 344) and ZF2 (aa349 to 398).
Expression Strictly regulated and tissue specific. Gene activity depends on several trans regulators and cis-acting regulatory elements located in the vicinity of the gene.
Localisation Nuclear.
Function Binds to the consensus sequence 5'-(A/T)GATA(A/G)-3'. Transcriptional activator which is expressed very early in hematopoiesis and plays a role in development and regulation of every early pluripotent hematopoietic precursor, but also of non hematopoietic embryonic stem cells. Early stages of erythroid differentiation depends of GATA2, but during maturation GATA2 expression decreases progressively at the benefit of GATA1.
Homology Member of the GATA family which contains 6 known members; only GATA1, GATA2 and GATA3 are involved in hematopoiesis.

Mutations

Germinal No known mutation.
Somatic In CML acute myeloid transformation (see below).

Implicated in

Entity Acute promyelocytic leukaemia
Disease GATA2 may be involved in APL leukemogenesis by physical interaction with the PML component of PML-RARa fusion or with the variant PLZF-RARa fusion, generated respectively by t(15;17) or t(11;17) translocation.
  
Entity Myelodysplasic syndrome
Disease GATA2 is expressed in MDS, but not in normal controls; the frequency of expression increases with the severity of dysplasia (100% in RAEB/RAEB-T).
  
Entity Myeloid transformation of chronic myelooid leukemia CML
Disease Out of 85 unselected cases of CML blast transformation, 9 showed a GATA2 mutation: 8 with a T-->G substitution at aa359 in ZF2 (L359V) and 1 with a 6 aa deletion (aa 341 to 346) in ZF1. All 9 transformations were myeloid, with a myeloblastic or monoblastic morphology. L359V leads to a gain of function of GATA2 protein.
  
Entity Aplastic anemia
Disease Hypothetical. In knockout mice, GATA2 haploinsufficiency leads to a decrease of hematopoietic stem cells number and efficiency. In human, GATA2 mRNA expression is largely reduced in patients with AA.
  

External links

Nomenclature
HGNC (Hugo)GATA2   4171
Entrez_Gene (NCBI)GATA2  2624  GATA binding protein 2
Cards
AtlasGATA2ID44160ch3q21
GeneCards (Weizmann)GATA2
Ensembl (Hinxton)ENSG00000179348 [Gene_View]  chr3:128198265-128207373 [Contig_View]  GATA2 [Vega]
AceView (NCBI)GATA2
Genatlas (Paris)GATA2
SOURCE (Stanford)NM_001145661 NM_001145662 NM_032638
Genomic and cartography
GoldenPath (UCSC)GATA2  -  3q21.3   chr3:128198265-128207373 -  3q21   [Description]    (hg19-Feb_2009)
EnsemblGATA2 - 3q21 [CytoView]
Mapping of homologs : NCBIGATA2 [Mapview]
OMIM137295   601626   614038   614172   614286   
Gene and transcription
Genbank (Entrez)AI524325 AK127845 AK314826 BC002557 BC015577
RefSeq transcript (SRS)NM_001145661 NM_001145662 NM_032638
RefSeq transcript (Entrez)NM_001145661 NM_001145662 NM_032638
RefSeq genomic (SRS)AC_000135 NC_000003 NC_018914 NG_029334 NT_005612 NW_001838883 NW_004078013
RefSeq genomic (Entrez)AC_000135 NC_000003 NC_018914 NG_029334 NT_005612 NW_001838883 NW_004078013
Consensus coding sequences : CCDS (NCBI)GATA2
Cluster EST : UnigeneHs.367725 [ SRS ] Hs.367725 [ NCBI ]
CGAP (NCI)Hs.367725
Alternative Splicing : Fast-db (Paris)GSHG0022036
Alternative Splicing GalleryENSG00000179348
Gene ExpressionGATA2 [ NCBI-GEO ]   GATA2 [ EBI - ARRAY_EXPRESS ]
Protein : pattern, domain, 3D structure
UniProt/SwissProtP23769 (SRS) P23769 (Uniprot)
NextProtP23769
With graphics : InterProP23769
Splice isoforms : SwissVarP23769(Swissvar)
Domaine pattern : Prosite (SRS)GATA_ZN_FINGER_1 (PS00344)    GATA_ZN_FINGER_2 (PS50114)   
Domaine pattern : Prosite (Expaxy)GATA_ZN_FINGER_1 (PS00344)    GATA_ZN_FINGER_2 (PS50114)   
Domains : Interpro (SRS)TF_GATA-1/2/3    Znf_GATA    Znf_NHR/GATA   
Domains : Interpro (EBI)TF_GATA-1/2/3    Znf_GATA    Znf_NHR/GATA   
Related proteins : CluSTrP23769
Domain families : Pfam (SRS)GATA (PF00320)   
Domain families : Pfam (Sanger)GATA (PF00320)   
Domain families : Pfam (NCBI)pfam00320   
Domain families : Smart (EMBL)ZnF_GATA (SM00401)  
DMDM2624
Blocks (Seattle)P23769
Human Protein AtlasENSG00000179348
HPRD00673
IPIIPI00012900   IPI00794907   IPI00944983   IPI00947465   
Protein Interaction databases
DIP (DOE-UCLA)P23769
IntAct (EBI)P23769
FunCoupENSG00000179348
REACTOMEGATA2
Protein Interaction Database2624
BioGRIDGATA2
InParanoidP23769
Interologous Interaction database P23769
IntegromeDBGATA2
Polymorphism : SNP, mutations, diseases
SNP Single Nucleotide Polymorphism (NCBI)GATA2
SNP (GeneSNP Utah)GATA2
SNP : HGBaseGATA2
Genetic variants : HAPMAPGATA2
Cancer Gene: CensusGATA2 
Somatic Mutations in Cancer : COSMICGATA2 
CONAN: Copy Number AnalysisGATA2 
Mutations and Diseases : HGMDGATA2
OMIM137295    601626    614038    614172    614286   
GENETests137295    601626    614038    614172    614286   
Disease Genetic AssociationGATA2
Huge Navigator GATA2 [HugePedia]  GATA2 [HugeCancerGEM]
Genomic VariantsGATA2  GATA2 [DGVbeta]
snp3D : Map Gene to Disease2624
General knowledge
Homologs : HomoloGeneGATA2
Homology/Alignments : Family Browser (UCSC)GATA2
Phylogenetic Trees/Animal Genes : TreeFamGATA2
Chemical/Protein Interactions : CTD2624
Chemical/Pharm GKB GenePA28585
Clinical trialGATA2
Cancer Resource (Charite)ENSG00000179348
Ontology : AmiGOnegative regulation of transcription from RNA polymerase II promoter  RNA polymerase II distal enhancer sequence-specific DNA binding  RNA polymerase II core promoter proximal region sequence-specific DNA binding transcription factor activity involved in positive regulation of transcription  enhancer sequence-specific DNA binding  urogenital system development  cell fate determination  embryonic placenta development  chromatin binding  sequence-specific DNA binding transcription factor activity  protein binding  nucleus  nucleoplasm  nucleolus  transcription, DNA-dependent  phagocytosis  blood coagulation  transcription factor binding  zinc ion binding  ventral spinal cord interneuron differentiation  cell differentiation in hindbrain  central nervous system neuron development  pituitary gland development  somatic stem cell maintenance  regulation of histone acetylation  eosinophil fate commitment  inner ear morphogenesis  protein complex  negative regulation of fat cell differentiation  positive regulation of erythrocyte differentiation  negative regulation of macrophage differentiation  positive regulation of megakaryocyte differentiation  negative regulation of Notch signaling pathway  positive regulation of angiogenesis  positive regulation of transcription from RNA polymerase II promoter  positive regulation of transcription from RNA polymerase II promoter  positive regulation of transcription from RNA polymerase II promoter  metal ion binding  cell maturation  neuron fate commitment  homeostasis of number of cells within a tissue  positive regulation of phagocytosis  definitive hemopoiesis  semicircular canal development  negative regulation of fat cell proliferation  C2H2 zinc finger domain binding  negative regulation of neural precursor cell proliferation  
Ontology : EGO-EBInegative regulation of transcription from RNA polymerase II promoter  RNA polymerase II distal enhancer sequence-specific DNA binding  RNA polymerase II core promoter proximal region sequence-specific DNA binding transcription factor activity involved in positive regulation of transcription  enhancer sequence-specific DNA binding  urogenital system development  cell fate determination  embryonic placenta development  chromatin binding  sequence-specific DNA binding transcription factor activity  protein binding  nucleus  nucleoplasm  nucleolus  transcription, DNA-dependent  phagocytosis  blood coagulation  transcription factor binding  zinc ion binding  ventral spinal cord interneuron differentiation  cell differentiation in hindbrain  central nervous system neuron development  pituitary gland development  somatic stem cell maintenance  regulation of histone acetylation  eosinophil fate commitment  inner ear morphogenesis  protein complex  negative regulation of fat cell differentiation  positive regulation of erythrocyte differentiation  negative regulation of macrophage differentiation  positive regulation of megakaryocyte differentiation  negative regulation of Notch signaling pathway  positive regulation of angiogenesis  positive regulation of transcription from RNA polymerase II promoter  positive regulation of transcription from RNA polymerase II promoter  positive regulation of transcription from RNA polymerase II promoter  metal ion binding  cell maturation  neuron fate commitment  homeostasis of number of cells within a tissue  positive regulation of phagocytosis  definitive hemopoiesis  semicircular canal development  negative regulation of fat cell proliferation  C2H2 zinc finger domain binding  negative regulation of neural precursor cell proliferation  
Other databases
Probes
Litterature
PubMed103 Pubmed reference(s) in Entrez
PubGeneGATA2
iHOPGATA2

Bibliography

Potentiation of GATA-2 activity through interactions with the promyelocytic leukemia protein (PML) and the t(15;17)-generated PML-retinoic acid receptor alpha oncoprotein.
Tsuzuki S, Towatari M, Saito H, Enver T.
Mol Cell Biol. 2000 Sep;20(17):6276-86.
PMID 10938104
 
Decreased expression of transcription factor GATA-2 in haematopoietic stem cells in patients with aplastic anaemia.
Fujimaki S, Harigae H, Sugawara T, Takasawa N, Sasaki T, Kaku M.
Br J Haematol. 2001 Apr;113(1):52-7.
PMID 11328281
 
GATA-1 and GATA-2 gene expression is related to the severity of dysplasia in myelodysplastic syndrome.
Fadilah SA, Cheong SK, Roslan H, Rozie-Hanisa M, Yen GK.
Leukemia. 2002 Aug;16(8):1563-5.
PMID 12145700
 
GATA transcription factors and hematological diseases.
Harigae H.
Tohoku J Exp Med. 2006 Sep;210(1):1-9. Review
PMID 16960339
 
GATA-1, -2 and -3 genes expression in bone marrow microenviroment with chronic aplastic anemia.
Wu X, Li Y, Zhu K, Wang Z, Chen S, Yang L.
Hematology. 2007 Aug;12(4):331-5.
PMID 17654061
 
Gain-of-function mutation of GATA-2 in acute myeloid transformation of chronic myeloid leukemia.
Zhang SJ, Ma LY, Huang QH, Li G, Gu BW, Gao XD, Shi JY, Wang YY, Gao L, Cai X, Ren RB, Zhu J, Chen Z, Chen SJ.
Proc Natl Acad Sci U S A. 2008 Feb 12;105(6):2076-81. Epub 2008 Feb 4.
PMID 18250304
 
REVIEW articlesautomatic search in PubMed
Last year publicationsautomatic search in PubMed

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Contributor(s)

Written01-2009Franck Viguié
Laboratoire de Cytogenetique - Service d'Hematologie Biologique, Hopital Hotel-Dieu - 75181 Paris Cedex 04, France

Citation

This paper should be referenced as such :
Viguié F . GATA2 (GATA binding protein 2). Atlas Genet Cytogenet Oncol Haematol. January 2009 .
URL : http://AtlasGeneticsOncology.org/Genes/GATA2ID44160ch3q21.html

This paper is referenced by INIST as such :
http://documents.irevues.inist.fr/bitstream/2042/44636/1/01-2009-GATA2ID44160ch3q21.pdf   [ Bibliographic record ]

© Atlas of Genetics and Cytogenetics in Oncology and Haematology
indexed on : Wed May 1 13:11:32 CEST 2013

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