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FLASH GENE

Symbol

LHX6

contributors: mct - updated : 07-07-2015

HGNC name	LIM homeobox 6
HGNC id	21735

FLASH GENE DNA RNA EXPRESSION PROTEIN DISORDER ANIMAL & CELL MODELS

EXPRESSION

Type

widely

expressed in	(based on citations)
organ(s)	System Organ level 1 Organ level 2 Organ level 3 Organ level 4 Level Pubmed Species Stage Rna symbol Digestive mouth palate     highly Homo sapiens Fetal   mouth tooth enamel   highly Homo sapiens Fetal Nervous brain forebrain cerebral cortex frontal   Reproductive female system uterus     highly   male system prostate     highly

cells

System Cell Pubmed Species Stage Rna symbol Digestive epithelial cell Homo sapiens Fetal

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)

Type Gene Modification Chromosome rearrangement Protein expression Protein 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