Gli3 Gene Summary [Mouse]
Enables DNA-binding transcription factor activity; chromatin binding activity; and sequence-specific DNA binding activity. Involved in several processes, including negative thymic T cell selection; regulation of alpha-beta T cell differentiation; and thymocyte apoptotic process. Acts upstream of or within several processes, including nervous system development; positive regulation of cell differentiation; and skeletal system morphogenesis. Located in axoneme and nuclear speck. Part of transcription repressor complex. Is expressed in several structures, including alimentary system; central nervous system; embryo mesenchyme; genitourinary system; and sensory organ. Used to study Greig cephalopolysyndactyly syndrome and Pallister-Hall syndrome. Human ortholog(s) of this gene implicated in Pallister-Hall syndrome; anodontia; polydactyly (multiple); and synostosis (multiple). Orthologous to human GLI3 (GLI family zinc finger 3). [provided by Alliance of Genome Resources, Jul 2025]
Details
| Type | Protein Coding |
| Official Symbol | Gli3 |
| Official Name | GLI-Kruppel family member GLI3 [Source:MGI Symbol;Acc:MGI:95729] |
| Ensembl ID | ENSMUSG00000021318 |
| Bio databases IDs | |
| Aliases | GLI-Kruppel family member GLI3 |
| Synonyms | ACLS, add, Bph, GCPS, GLI3-190, GLI3FL, GLI family zinc finger 3, GLI-Kruppel family member GLI3, PAP-A, PAPA1, PAPB, Pdn, PHS, PPDIV, Xt |
| Species | Mouse, Mus musculus |
| Orthologies |
Protein Domains
A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in mouse Gli3 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- 14-3-3 binding domain
- zinc finger DNA binding domain
- transcription regulator
- nucleic acid binding
- beta-catenin binding
- histone deacetylase binding
- chromatin binding
- protein binding
- sequence-specific DNA binding
- transcription repression domain
- binding protein
- processing determinant domain
- double-stranded DNA binding
- Zinc-finger double domain
- SYGH motif
- zinc finger domain
- Pka phosphorylation site
- transcription factor activity
- histone acetyltransferase binding
Pathways
Biological processes and signaling networks where the Gli3 gene in mouse plays a role, providing insight into its function and relevance in health or disease.
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
- prostate cancer
- age-related macular degeneration
- postaxial polydactyly type B
- GLI3-related disorder
- postaxial polydactyly type A1
- pallister-hall syndrome
- Greig cephalopolysyndactyly syndrome
- ulcerative colitis
- postaxial polydactyly type A1/B
- acute myeloid leukemia
phenotypes
- Harderian gland hyperplasia
- abnormal anterior cardinal vein morphology
- abnormal axial skeleton morphology
- abnormal basisphenoid bone morphology
- abnormal bone mineralization
- abnormal brain morphology
- abnormal cerebellar Purkinje cell layer
- abnormal cerebellum morphology
- abnormal cerebral aqueduct morphology
- abnormal cerebral cortex morphology
- abnormal cerebral hemisphere morphology
- abnormal choroid plexus morphology
- abnormal colon morphology
- abnormal cranial ganglia morphology
- abnormal craniofacial development
- abnormal craniofacial morphology
- abnormal cranium morphology
- abnormal digit development
- abnormal digit morphology
- abnormal ear development
- abnormal ear position
- abnormal embryonic autopod plate morphology
- abnormal embryonic tissue morphology
- abnormal endochondral bone ossification
- abnormal epidermal layer morphology
- abnormal epidermis stratum basale morphology
- abnormal eye development
- abnormal eye morphology
- abnormal facial morphology
- abnormal folding of telencephalic vesicles
- abnormal forebrain morphology
- abnormal forehead morphology
- abnormal hallux morphology
- abnormal hindbrain morphology
- abnormal hindlimb morphology
- abnormal hippocampal fornix morphology
- abnormal hippocampus development
- abnormal hippocampus pyramidal cell layer
- abnormal hypothalamus morphology
- abnormal inferior colliculus morphology
- abnormal interfrontal bone morphology
- abnormal intestine morphology
- abnormal kidney morphology
- abnormal lambdoid suture morphology
- abnormal laryngeal cartilage morphology
- abnormal lateral ventricle morphology
- abnormal left lung morphology
- abnormal limb bone morphology
- abnormal limb bud morphology
- abnormal limb morphology
- abnormal long bone morphology
- abnormal mammary gland bud morphology
- abnormal mammary line morphology
- abnormal maxilla morphology
- abnormal medulla oblongata morphology
- abnormal melanoblast morphology
- abnormal metacarpal bone morphology
- abnormal metatarsal bone morphology
- abnormal midbrain morphology
- abnormal midbrain-hindbrain boundary morphology
- abnormal nail morphology
- abnormal nasal cavity morphology
- abnormal nasal placode morphology
- abnormal nasal septum morphology
- abnormal neural tube morphology
- abnormal neurocranium morphology
- abnormal neuronal migration
- abnormal nose morphology
- abnormal olfactory epithelium morphology
- abnormal olfactory nerve morphology
- abnormal olfactory tract morphology
- abnormal optic chiasm morphology
- abnormal optic cup morphology
- abnormal optic stalk morphology
- abnormal optic vesicle formation
- abnormal orbitosphenoid bone morphology
- abnormal otic capsule morphology
- abnormal otic vesicle development
- abnormal palatine bone horizontal plate morphology
- abnormal pericardium morphology
- abnormal phalanx morphology
- abnormal pharyngeal arch morphology
- abnormal pubis morphology
- abnormal radial glial cell morphology
- abnormal retina morphology
- abnormal rhombomere morphology
- abnormal right lung accessory lobe morphology
- abnormal right lung caudal lobe morphology
- abnormal right lung middle lobe morphology
- abnormal skeleton morphology
- abnormal spine curvature
- abnormal sternum morphology
- abnormal stratification in cerebral cortex
- abnormal superior colliculus morphology
- abnormal superior semicircular canal morphology
- abnormal telencephalon development
- abnormal thalamus morphology
- abnormal tooth development
- abnormal vertebrae morphology
- abnormal vertebral arch morphology
- abnormal vibrissa morphology
- abnormal vibrissa number
- abnormal xiphoid process morphology
- absent adrenal gland
- absent anterior commissure
- absent corpus callosum
- absent epiglottis
- absent hippocampal commissure
- absent lateral semicircular canal
- absent neurocranium
- absent olfactory bulb
- absent optic nerve
- absent pineal gland
- absent radius
- absent tibia
- acoria
- anal atresia
- anophthalmia
- basisphenoid bone foramen
- belly spot
- bleb
- bowed fibula
- bowed radius
- bowed tibia
- bowed ulna
- brachyphalangia
- camptomelia
- cleft palate
- cleft upper lip
- decreased lung weight
- decreased nipple number
- delayed neural tube closure
- ectopic adrenal gland
- ectopic ovary
- edema
- embryonic growth retardation
- embryonic lethality
- embryonic lethality before implantation complete penetrance
- embryonic lethality during organogenesis incomplete penetrance
- encephalomeningocele
- enlarged cranium
- enlarged first pharyngeal arch
- enlarged tectum
- exencephaly
- eyelids fail to open
- eyelids open at birth
- failure of endochondral bone ossification
- fused carpal bones
- fused tarsal bones
- fusion of vertebral arches
- hemimelia
- hemorrhage
- herniated abdominal wall
- hydrocephaly
- hypopigmentation
- incomplete rostral neuropore closure
- increased diameter of humerus
- increased diameter of radius
- increased diameter of ulna
- increased embryo size
- increased forebrain size
- increased total retina thickness
- interdigital webbing
- microphthalmia
- neonatal lethality complete penetrance
- omphalocele
- perinatal lethality
- perinatal lethality complete penetrance
- polydactyly
- polyphalangy
- polysyndactyly
- postnatal lethality
- postnatal lethality complete penetrance
- preaxial polydactyly
- premature lambdoid suture closure
- prenatal lethality
- prenatal lethality incomplete penetrance
- reduced cerebellar foliation
- respiratory distress
- sacral vertebral fusion
- short femur
- short fibula
- short humerus
- short limbs
- short nasal septum
- short radius
- short sternum
- short tibia
- short ulna
- single kidney
- skin edema
- small basisphenoid bone
- small lung
- split sternal manubrium
- spongiform encephalopathy
- sternebra fusion
- syndactyly
- synostosis
- thick apical ectodermal ridge
- thick nasal septum
- thin cerebral cortex
- vertebral fusion
- wavy neural tube
- wide metopic suture
- wide sagittal suture
role in cell
- differentiation
- proliferation
- apoptosis
- migration
- cellular infiltration by
- cell death
- accumulation
- expression in
- quantity
- activation in
Subcellular Expression
Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
- Nucleus
- motile cilia
- intracellular membrane-bounded organelle
- ciliary tip
- Cytoplasm
- cilia
- axonemes
- cytosol
- nucleoplasm
- nucleoli
- nuclear speckles
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the mouse Gli3 gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- frontal suture morphogenesis
- lambdoid suture morphogenesis
- anterior/posterior pattern specification
- nose morphogenesis
- forebrain dorsal/ventral pattern formation
- axon guidance
- embryonic digestive tract morphogenesis
- tongue development
- artery development
- sagittal suture morphogenesis
- proximal/distal pattern formation
- embryonic digit morphogenesis
- optic nerve morphogenesis
- neuroblast proliferation
- embryonic digestive tract development
- negative regulation of transcription, DNA-dependent
- positive regulation of transcription, DNA-dependent
- in utero embryonic development
- thymocyte apoptotic process
- mammary gland specification
- positive regulation of protein import into nucleus
- smoothened signaling pathway
- positive regulation of neuroblast proliferation
- cell differentiation involved in kidney development
- anterior semicircular canal development
- forebrain radial glial cell differentiation
- smoothened signaling pathway involved in dorsal/ventral neural tube patterning
- lateral semicircular canal development
- negative regulation of stem cell proliferation
- protein import into nucleus
- limb morphogenesis
- protein processing
- negative thymic T cell selection
- negative regulation of alpha-beta T cell differentiation
- layer formation in cerebral cortex
- palate development
- chondrocyte differentiation
- negative regulation of neuron differentiation
- smoothened signaling pathway involved in ventral spinal cord interneuron specification
- smoothened signaling pathway involved in spinal cord motor neuron cell fate specification
- odontogenesis of dentin-containing tooth
- alpha-beta T cell differentiation
- positive regulation of osteoblast differentiation
- positive regulation of transcription from RNA polymerase II promoter
- positive regulation of alpha-beta T cell differentiation
- negative regulation of transcription from RNA polymerase II promoter
- heart development
- stem cell proliferation
- osteoblast differentiation
- lung development
- oligodendrocyte differentiation
- T cell differentiation in thymus
- embryonic neurocranium morphogenesis
- regulation of transcription, DNA-dependent
- lateral ganglionic eminence cell proliferation
- hippocampus development
- hindgut morphogenesis
- developmental growth
- negative regulation of smoothened signaling pathway
- regulation of transcription from RNA polymerase II promoter
- negative regulation of canonical Wnt receptor signaling pathway
- melanocyte differentiation
- camera-type eye morphogenesis
- metanephros development
- branching involved in ureteric bud morphogenesis
- negative regulation of apoptotic process
- negative regulation of chondrocyte differentiation
- positive regulation of chondrocyte differentiation
- vocalization behavior
Cellular Component
Where in the cell the gene product is active
- nucleus
- cytoplasm
- transcriptional repressor complex
- nuclear speck
- intracellular membrane-bounded organelle
- cilium
- cytosol
- nucleolus
- axoneme
- nucleoplasm
Molecular Function
What the gene product does at the molecular level
- histone acetyltransferase binding
- sequence-specific DNA binding transcription factor activity
- protein binding
- histone deacetylase binding
- metal ion binding
- RNA polymerase II core promoter proximal region sequence-specific DNA binding
- mediator complex binding
- RNA polymerase II regulatory region sequence-specific DNA binding
- beta-catenin binding
- chromatin binding
- sequence-specific DNA binding RNA polymerase II transcription factor activity