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FLASH GENE
Symbol ZFPM2 contributors: mct/npt - updated : 02-05-2018
HGNC name zinc finger protein, multitype 2
HGNC id 16700
Corresponding disease
CTHD1 conotruncal heart defects 1
SRXY9 46XY sex reversal 9, ZFPM2-related
Location 8q23.1      Physical location : 106.331.146 - 106.816.765
Synonym name
  • friend of GATA2
  • modulator of transcription factor GATA4
  • zinc finger protein 89B
  • Synonym symbol(s) FOG2, DIH3, MGC129663, MGC129664, ZNF89B, hFOG-2, SRXY9, ZC2HC11B
    DNA
    TYPE functioning gene
    STRUCTURE 485.68 kb     8 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status provisional
    Map cen - D8S270 - ATP6V0D1 - D8S1789 - D8S1784 - D8S556 - ZFPM2 - D8S100 - D8S1830 - SDC2 - D8S257 - qter
    Authors Gene Map (98)
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    8 - 4507 128 1151 - 1999 10438528
    7 - 4348 - 1098 . predominately in the embryonic and adult heart 1999 10438528
  • variant 2 (FOG2s)
  • lacking the FOG repression
  • can bind GATA4 avidly, but is unable to repress GATA4-mediated activation of cardiac-restricted gene promoters
  • 8 - 4656 - 1019 - 1999 10438528
    EXPRESSION
    Type widely
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularheart   predominantly
    Nervousbrain     Homo sapiens
    Reproductivefemale systemuterus    Homo sapiens
     male systemtestis   
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Muscularsmooth    Homo sapiens
    cells
    SystemCellPubmedSpeciesStageRna symbol
    cardiovascularheart
    cell lineage hematopoietic cell lines
    cell lines
    fluid/secretion
    at STAGE
    physiological period embryo, pregnancy
    Text developing heart (myocytes), neuroepithelium and urogenital ridge
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • one N terminal interaction domains for GATA4, CTBP2 and a transcriptional repression motif
  • eight zinc finger motifs
  • a LXCXE pRB-binding site of ZFPM2 is involved in adipogenesis
  • conjugated MetalloP
    HOMOLOGY
    interspecies homolog to murine Fog2
    Homologene
    FAMILY
  • FOG (Friend of GATA) family
  • CATEGORY transcription factor
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm
    intracellular,nucleus,nucleoplasm
    basic FUNCTION
  • cardiac nuclear hormone receptor corepressor protein (Rouf 2008)
  • development of coronary vasculature from epicardium
  • with GATA4 in the early pulmonary mesenchyme participate in patterning the secondary bronchus of the accessory lobe) (Ackerman 2007)
  • its function is required for correct progression of preadipocyte differentiation . plays a role in the regulation of early adipogenesis
  • plays a role in the regulation of early adipogenesis
  • transcriptional cofactor involved mostly in cardiac function
  • ZFPM2 plays a role in cardiac development by acting as a transcriptional cofactor that interacts with GATA4
  • unlike GATA1, which inhibits cell division, ZFPM2 promotes proliferation
  • CELLULAR PROCESS nucleotide, transcription
    PHYSIOLOGICAL PROCESS cardiovascular , development , reproduction/sex
    text folliculogenesis
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • activator of GATA4 gene expression during cardiac development and other GATA genes expression, bind to the N-terminal zinc finger of GATA4 and, via this interaction, repress GATA4-mediated transcriptional activation of various cardiac promoters
  • corepressor of GATA5 through interaction with CTPB2
  • bind to GATA factors and function as transcriptional co-repressors in specific cell and promoter contexts
  • intreacting with SERCA2 (SERCA2 is an important target and increased ZFPM2 expression may contribute to a decline in cardiac function in end-stage heart failure by impaired T3 signaling) (Rouf 2008)
  • LHX9 gene represents the first direct target of the GATA4/ZFPM2 repressor complex in cardiac development
  • cardiac expression of TNNT1 strictly depends on the physical interaction between GATA4-ZFPM2 in the myocardium of both atria and ventricles
  • GATA4 may alter the activity of ZFPM2 by influencing its SUMOylation status
  • sex determination requires interaction between the transcription factor GATA4 and its cofactor ZFPM2
  • ZFPM2 is a multi-zinc finger protein that binds the transcriptional activator GATA4 and modulates GATA4-mediated regulation of target genes during heart development
  • ZFPM2/NuRD interaction is required for cardiomyocyte proliferation by directly down-regulating the cell cycle inhibitor CDKN1A during heart development
  • novel function of hepatic ZFPM2 in insulin sensitivity and lipid metabolism through PPARA
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) SRXY9 , CTHD1
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional germinal mutation      
    in some sporadic cases of tetralogy of Fallot
    constitutional   translocation   loss of function
    truncated transcript associated with an atrial septal defect and gonadal dysgenesis in 1 patient
    constitutional     --over  
    in cardiomyopathy (Rouf 2008)
    constitutional germinal mutation      
    result in primary pulmonary and diaphragmatic defects (Ackerman 2005)
    Susceptibility to glioma
    Variant & Polymorphism insertion/deletion
  • insertion-deletion polymorphism rs71305152 was significantly associated with gliomas
  • Candidate gene to cardiac outflow tract defects
    Marker
    Therapy target
    ANIMAL & CELL MODELS