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FLASH GENE
Symbol LHX6 contributors: mct - updated : 07-07-2015
HGNC name LIM homeobox 6
HGNC id 21735
Location 9q33.2      Physical location : 124.964.859 - 124.991.019
Synonym name LIM homeodomain protein 6.1
Synonym symbol(s) LHX6.1, MGC119542, MGC119544, MGC119545
DNA
TYPE functioning gene
STRUCTURE 26.31 kb     10 Exon(s)
10 Kb 5' upstream gene genomic sequence study
regulatory sequence
text structure highly conserved sequence in the LHX6 promoter, which appears to mediate the direct activation of LHX6 by NKX2.1
MAPPING cloned Y linked N status provisional
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
10 splicing 3377 - 392 - 2006 16732332
9 splicing 3273 - 377 - 2006 16732332
a shorter and distinct C-terminus as a result of a frameshit in the coding region
9 - 3306 - 366 - 2006 16732332
9 - 3394 - 363 - 2006 16732332
7 - 3603 - 176 - 2006 16732332
EXPRESSION
Type widely
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Digestivemouthpalate  highly Homo sapiensFetal
 mouthtoothenamel highly Homo sapiensFetal
Nervousbrainforebraincerebral cortexfrontal 
Reproductivefemale systemuterus  highly
 male systemprostate  highly
cells
SystemCellPubmedSpeciesStageRna symbol
Digestiveepithelial cell Homo sapiensFetal
cell lineage
cell lines
fluid/secretion
at STAGE
physiological period embryo
Text brain
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