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FLASH GENE
Symbol LMO2 contributors: mct - updated : 10-09-2016
HGNC name LIM domain only 2 (rhombotin-like 1)
HGNC id 6642
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • two N-terminal cysteine-rich LIM/double zinc finger motifs (LIM domains), for binding to the ubiquitous nuclear adaptor protein LDB1
  • a consensus-binding site for a small family of basic leucine zipper proteins containing a proline and acidic amino acid-rich (PAR) domain
  • four zinc-finger domains
    • HOMOLOGY
    Homologene
    FAMILY
  • Zinc binding protein family
    • CATEGORY transcription factor , protooncogene
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,nucleus
    basic FUNCTION
  • required for yolk sac erythropoiesis
  • critical for the regulation of red blood cell development through interactions with TAL1
  • acting differently depending on the stage of hematopoietic development
  • essential for the regulation of hematopoietic stem cells and formation of erythroid cells
  • T-cell oncogenic protein first recognized by gene activation through chromosomal translocations, but it is also responsible for leukaemias arising as secondary (
  • key regulator of hematopoietic stem cell development whose ectopic expression in T cells leads to the onset of acute lymphoblastic leukemia
  • transcriptional regulator that is essential for normal haematopoietic development
  • transcription factor that plays a critical role in both hematopoietic development and vascular remodeling
  • is a component of multisubunit DNA-binding transcription factor complexes that regulate gene expression in hematopoietic stem and progenitor cell development
  • novel function of LMO2 involving in the regulatory hierarchy of UBA6-USE1-FAT10ylation pathway by targeting the E1 enzyme UBA6
    • CELLULAR PROCESS nucleotide, transcription
    PHYSIOLOGICAL PROCESS development
    PATHWAY
    metabolism
    signaling
    a component
  • association with LMO1 (and others) for the formation of multimeric DNA-binding complex for the generation of hematopoietic lineages and angiogenesis (in mice)
  • part of the DNA-binding complex containing LMO2, LDB1, TAL1, and GATA1
    • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • interacting with BEX2
  • also interacting with NHLH2
  • modulating the transcriptional activity of HEN1
  • interacted strongly to LDB1, which was required for the localization to the nucleus
  • probably promotes angiogenesis by up-regulation of FGF2 expression and thereby consequently influences progression of infantile haemangioma
  • oncogenic LCK may activate LMO2 promoter through direct interaction
  • displayed histone H3K9-me1/2 demethylase activity and induced leukemogenic oncogene LMO2 expression via a synergistic interaction with CREBBP
  • TAL1 associates with GATA1, GATA2, lim-only protein LMO2, and LDB1 in a multimeric complex to regulate erythroid transcription
  • GATA2 and LMO2 cooperatively regulate VEGFA-induced angiogenesis and lymphangiogenesis via NRP2
  • coexpression of full-length, wild-type LDB1 increased LMO2 steady-state abundance, whereas coexpression of mutant proteins deficient in LMO2 binding compromised LMO2 stability
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral   translocation    
    acute lymphoblastic leukemia (T-ALL) with translocations t(11;14)(p13;q11), t(7;11)(p35;p13)
    tumoral       loss of function
    in renal clear cell carcinomas with poor prognosis
    tumoral fusion      
    t(14;18)/IGH-BCL2 fusion in diffuse large B-cell lymphoma
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker promising marker for predicting a better prognosis in pancreatic cancer
    Therapy target
    SystemTypeDisorderPubmed
    cancerhemopathy 
    assembly of LMO2 into complexes, via direct LDB1 interaction, is a potential molecular target that could be exploited in LMO2-driven leukemias resistant to existing chemotherapy regimens
    ANIMAL & CELL MODELS