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FLASH GENE
Symbol LHX6 contributors: mct - updated : 07-07-2015
HGNC name LIM homeobox 6
HGNC id 21735
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • two tandem LIM domains
  • a central homeo domain a homeodomain that regulate its activity both in cis and through interaction with cell-specific factors
    • HOMOLOGY
    interspecies homolog to murine Lhx6
    Homologene
    FAMILY
  • LIM domain containing protein family
    • CATEGORY regulatory , transcription factor
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,nucleus
    basic FUNCTION
  • may function as a transcriptional regulator
  • may be involved in the control of differenciation and development of neural and lymphoid cells
  • its activity is required for the normal tangential and radial migration of GABAergic interneurons in the cortex
  • redundant activities of the LIM homeodomain proteins LHX6 and LHX8 are critical for craniofacial development and patterning of mammalian dentition
  • plays an important role in the maturation of cortical interneurons and the formation of inhibitory circuits in the mammalian cortex
  • LHX6 and the PITX2 homeodomain transcription factor have overlapping expression patterns during tooth and craniofacial development
  • acts as a transcriptional repressor and represses the expression of several genes involved in odontogenesis
  • regulates cell proliferation in the cervical loop and promotes cell differentiation in the anterior region of the incisor
  • is required for normal odontoblast, bone, and root development
  • a putative transcriptional regulator that controls the differentiation and development of neural and lymphoid cells
  • is a putative tumour suppressor gene with epigenetic silencing in lung cancer
  • regulates parvalbumin and somatostatin neuron development
    • CELLULAR PROCESS nucleotide, transcription, regulation
    PHYSIOLOGICAL PROCESS development , nervous system
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA binding
    RNA
    small molecule metal binding,
  • Zn2+
    • protein
  • interacts with LDB1
  • directly interacts with PITX2 to inhibit PITX2 transcriptional activities and activation of multiple promoters, and LHX6 acts to attenuate PITX2 autoregulation
  • directly binds to an ARX enhancer and to an intronic CXCR7 enhancer that remains active in mature interneurons
  • likely LHX6 and LHX8 negatively regulate CDKN1C expression in the prospective palate area to allow adequate levels of cell proliferation and thereby promote normal palate development
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --low  
    by hypermethylation in head and neck squamous cell carcinoma and colon carcinoma
    constitutional     --low  
    in schizophrenia
    tumoral     --low  
    by hypermethylation in lung cancer
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • amelogenin and ameloblastin expression is reduced and/or delayed in the Lhx6 null mice, potentially resulting from defects in dentin deposition and ameloblast differentiation