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Symbol PROX1 contributors: SGE/npt - updated : 25-02-2015
HGNC name prospero-related homeobox 1
HGNC id 9459
Corresponding disease
HLHS3 hypoplastic left heart syndrome, PROX1 related
Location 1q32.3      Physical location : 214.161.859 - 214.209.760
Synonym name
  • homeobox prospero-like protein PROX1
  • prospero homeobox 1
  • Synonym symbol(s) PROX-1
    TYPE functioning gene
    STRUCTURE 53.58 kb     5 Exon(s)
    Genomic sequence alignment details
    10 Kb 5' upstream gene genomic sequence study
    regulatory sequence Promoter
    text structure
  • expression is controlled by a hypoxia-response element (HRE) at the PROX1 promoter/regulatory region and HIF1A/HIF2A
  • MAPPING cloned Y linked N status provisional
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    5 - 8178 - 737 - 2009 19706680
    5 - 8505 - 737 - 2009 19706680
       expressed in (based on citations)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Lymphoid/Immunelymph node    
    Nervousbrainlimbic systemhippocampusAmmons horn  Mus musculus
     brainlimbic systemhippocampusdentate gyrus  Mus musculus
    Visualeyelens  highly
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Blood / Hematopoieticbone marrow   
    SystemCellPubmedSpeciesStageRna symbol
    Cardiovascularendothelial cell
    Nervouspyramidal cell Mus musculus
    cell lineage
    cell lines
    at STAGE
    physiological period embryo
    Text lens
  • helix-turn-helix domain
  • prospero homeobox family
  • CATEGORY transcription factor
    SUBCELLULAR LOCALIZATION     intracellular
    basic FUNCTION
  • role in cellular proliferation and in the lens fiber development and lymphatic system
  • specific regulator of the development of the lymphatic system and lymphatic endothelial-cell-specific transcripts
  • controlling progenitor cell proliferation and horizontal cell genesis in the retina
  • plays central roles in the differentiation of blood vascular endothelial cells (BECs) into lymphatic endothelial cells (LECs), and subsequently in the maturation and maintenance of lymphatic vessels (Harada 2009)
  • master regulator for the development of lymphatic vasculature and the induction of lymphangiogenesis (Pan 2009)
  • inhibits NOTCH1 gene expression to control the balance between neural progenitor cells (NPCs) self-renewal and neuronal differentiation
  • occupies promoters metabolic genes and inhibits the activity of the ESRRA/PPARGC1A complex (Charest-Marcotte 2010)
  • having a role in the control of energy homeostasis (Charest-Marcotte 2010)
  • is regulated by canonical WNT signaling and has a stage-specific role in adult hippocampal neurogenesis
  • acts as a postmitotic cell fate determinant for dentate gyrus granule cells over the CA3 pyramidal cell fate and is crucial for maintenance of the granule cell identity throughout the life
  • its overexpression has the ability to reprogram venous endothelium but not early arterial endothelial cells
  • PROX1-mediated epigenetic repression is involved in the physiologically essential negative feedback inhibition of CYP7A1 transcription by bile acids
  • controls binary fate decisions between motor neurons (MNs) and V2 interneurons in neural progenitor cells (NPCs) via direct repression of OLIG2 gene regulatory elements
  • functions as a tumor suppressor gene in oral carcinogenesis
  • critical intrinsic regulator of neurogenesis in the embryonic CNS and adult dentate gyrus (DG) of the hippocampus, acting in multiple ways and instructed by extrinsic cues and intrinsic factors
  • CELLULAR PROCESS nucleotide, transcription
    a component
    DNA binding
    small molecule
  • CDKN1B
  • CDKN1C
  • interacting with HOXD8 (transcriptional networks of PROX1 and HOXD8 play important roles in the maturation and maintenance of lymphatic vessels) (Harada 2009)
  • target for SUMO1 and sumoylation of PROX1 controls its ability to induce VEGFR3 expression and lymphatic phenotypes in endothelial cells (Pan 2009)
  • new negative regulator of IFN-gamma expression in T cells (Wang 2008)
  • interacting with HDAC3 (sumoylation of PROX1 reduces its interaction with HDAC3 and as a result downregulates its corepressor activity) (Shan 2008)
  • physically and functionally interacts with NR2F2 to specify lymphatic endothelial cell fate (Lee 2009)
  • SOX1 regulates the size of the cortical neural progenitor (NP) pool via suppression of PROX1-mediated neurogenic cell divisions
  • could negatively regulate NR1I2 signals in some tumor cells, such as hepatocellular carcinoma (HCC) cells, where both NR1I2 and PROX1 are expressed
  • TP53 directly regulates MAF and PROX1, two important transcription factors controlling differentiation in the ocular lens
  • interacts with KDM1A to recruit the repressive KDM1A/NuRD complex to CYP7A1 promoter and co-represses transcription through epigenetic mechanisms
  • transcription repressor and downstream target of proneural genes, suppresses OLIG2 expression and therefore controls ventral spinal cord patterning
  • cell & other
    induced by hypoxia
    Other regulated by NR2F2 (NR2F2 regulates the functions of PROX1 in lymphatic endothelial cells through direct interaction)(Yamazaki 2009)
    corresponding disease(s) HLHS3
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional       loss of function
    in adult-onset obesity with abnormal lymph leakage from mispatterned and ruptured lymphatic vessels
    tumoral     --low  
    in primary lymphoid malignancies by aberrant DNA methylation
    constitutional     --low  
    reduced expression of PROX1 by cis-regulatory variants results in altered beta-cell insulin secretion and thereby confers susceptibility to type 2 diabetes
    Variant & Polymorphism
    Candidate gene
  • should be considered as a potential candidate gene for cases of hypoplastic left heart(Gill 2009)
  • Marker
    Therapy target