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Six2 Gene Detail
Summary
  • Symbol
    Six2
  • Name
    sine oculis-related homeobox 2
  • Feature Type
    protein coding gene
  • IDs
    MGI:102778
    NCBI Gene: 20472
  • Alliance
  • Transcription Start Sites
    3 TSS
Location &
Maps
more
  • Sequence Map
    Chr17:85991705-85995702 bp, - strand
    From Ensembl annotation of GRCm39
  • View this region in JBrowse
  • Genome Browsers
  • Genetic Map
    Chromosome 17, 55.72 cM
  • Mapping Data
    3 experiments
Strain
Comparison
more
  • SNPs within 2kb
    178 from dbSNP Build 142
  • Strain Annotations
    19
  • RFLP
For selected strains:
Strain Gene Model ID Feature Type Coordinates Select Strains
C57BL/6J MGI_C57BL6J_102778
protein coding gene Chr17:85970276-85995702 (-)
129S1/SvImJ ENSMUSG00200021939
protein coding gene Chr17:81394974-81398980 (-)
A/J ENSMUSG00195027987
protein coding gene Chr17:82768728-82772713 (-)
AKR/J ENSMUSG00220016259
protein coding gene Chr17:81719021-81723035 (-)
BALB/cJ ENSMUSG00180025544
protein coding gene Chr17:82440862-82444857 (-)
C3H/HeJ ENSMUSG00175006806
protein coding gene Chr17:81706869-81710852 (-)
C57BL/6NJ ENSMUSG00215014806
protein coding gene Chr17:81434178-81438171 (-)
CAROLI/EiJ MGP_CAROLIEiJ_G0021933
protein coding gene Chr17:81289200-81293262 (-)
CAST/EiJ ENSTCUG00005011564
protein coding gene Chr17:82261594-82265700 (-)
CBA/J ENSMUSG00210022153
protein coding gene Chr17:81565662-81569649 (-)
DBA/2J ENSMUSG00185019476
protein coding gene Chr17:84380643-84384636 (-)
FVB/NJ ENSMUSG00205005726
protein coding gene Chr17:82188790-82192790 (-)
JF1/MsJ ENSUMUG00000008879
protein coding gene Chr17:84109014-84113176 (-)
LP/J ENSMUSG00230009472
protein coding gene Chr17:85413486-85417473 (-)
NOD/ShiLtJ ENSMUSG00190009361
protein coding gene Chr17:81464151-81468148 (-)
NZO/HlLtJ ENSMUSG00225030111
protein coding gene Chr17:87671269-87675266 (-)
PWK/PhJ ENSLUMG00010031313
protein coding gene Chr17:81324938-81329023 (-)
SPRET/EiJ ENSMSPG00010020014
protein coding gene Chr17:81906091-81910207 (-)
WSB/EiJ ENSIUOG00005020351
protein coding gene Chr17:82380027-82384016 (-)



Homology
more
  • Human Ortholog
    SIX2, SIX homeobox 2
  • Vertebrate Orthologs
    4
Vertebrate Orthology Source
Alliance of Genome Resources
  • Human Ortholog
    SIX2, SIX homeobox 2
  • Links
    NCBI Gene ID: 10736
    UniProt: Q9NPC8

  • Chr Location
    2p21; chr2:45005182-45009452 (-)  GRCh38

Human Diseases
more
  • Diseases
    1 with Six2 mouse models; 1 with human SIX2 associations

Human Disease Mouse Models
      
IDs
View 1 model
      
IDs
Click on a disease name to see all genes associated with that disease.

  • Mutations/Alleles
    2 with disease annotations
  • References
    3 with disease annotations
Mutations,
Alleles, and
Phenotypes
less
  • Phenotype Summary
    17 phenotypes from 4 alleles in 6 genetic backgrounds
    49 phenotypes from multigenic genotypes
    10 images
    71 phenotype references
Phenotype Overview

adipose tissue
behavior/neurological
cardiovascular system
cellular
craniofacial
digestive/alimentary system
embryo
endocrine/exocrine glands
growth/size/body
hearing/vestibular/ear
hematopoietic system
homeostasis/metabolism
integument
immune system
limbs/digits/tail
liver/biliary system
mortality/aging
muscle
nervous system
pigmentation
renal/urinary system
reproductive system
respiratory system
skeleton
taste/olfaction
neoplasm
vision/eye

Click cells to view annotations.
Mice homozygous for disruptions in this gene die shortly after birth and exhibit abnormal kidney development. Abnormalities include small kidney, lack of ureteric bud branches throughout the kidney, increased apoptosis and premature and arrested nephron development.
Gene Ontology
(GO)
Classifications
less
  • All GO Annotations
  • GO References
Molecular Function

carbohydrate derivative binding
cytoskeletal protein binding
DNA binding
enzyme regulator
hydrolase
ligase
lipid binding
oxidoreductase
RNA binding
signaling receptor activity
signaling receptor binding
transcription
transferase
transporter
Biological Process

carbohydrate derivative metabolism
cell differentiation
cell population proliferation
cellular component organization
DNA-templated transcription
establishment of localization
homeostatic process
immune system process
lipid metabolic process
programmed cell death
protein metabolic process
response to stimulus
signaling
system development
Cellular Component

cell projection
cytoplasmic vesicle
cytoskeleton
cytosol
endoplasmic reticulum
endosome
extracellular region
Golgi apparatus
mitochondrion
membraneless organelle
nucleus
organelle envelope
organelle lumen
plasma membrane
protein-containing complex
synapse
vacuole
Click cells to view annotations.
Expression
less
Expression Overview

early conceptus
embryo ectoderm
embryo endoderm
embryo mesoderm
embryo mesenchyme
extraembryonic component
alimentary system
auditory system
branchial arches
cardiovascular system
connective tissue
endocrine system
exocrine system
hemolymphoid system
integumental system
limbs
liver and biliary system
musculoskeletal system
nervous system
olfactory system
reproductive system
respiratory system
urinary system
visual system
Click cells to view annotations.


  • Assay Results
  • Tissues
  • cDNA Data
  • Literature Summary
  • Comparison Matrix
  • Sequences &
    Gene Models
    less
    Representative SequencesLengthStrain/SpeciesFlank
    genomic ENSMUSG00000024134 Ensembl Gene Model | MGI Sequence Detail 3998 C57BL/6J ±  kb
    transcript ENSMUST00000163568 Ensembl | MGI Sequence Detail 2119 Not Applicable  
    polypeptide ENSMUSP00000125871 Ensembl | MGI Sequence Detail 296 Not Applicable  
    For the selected sequence
    Protein
    Information
    less
    Molecular
    Reagents
    less
    • All nucleic 56
      Genomic 1
      cDNA 16
      Primer pair 18
      Other 21
      Antibodies 13

      Microarray probesets 3
    Other
    Accession IDs
    less
    MGD-MRK-19686
    References
    more
    • Summaries
      All 341
      Developmental Gene Expression 257
      Diseases 3
      Gene Ontology 25
      Phenotypes 71
    • Earliest
      J:23775 Oliver G, et al., Homeobox genes and connective tissue patterning. Development. 1995 Mar;121(3):693-705
    • Latest
      J:391359 Palhazi B, et al., The absence of Trim28 in nephron progenitors results in impaired kidney development and function. Development. 2026 Aug 1;153(15):dev205188

    Contributing Projects:
    Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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    Funding Information
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    last database update
    09/08/2026
    MGI 6.24
    The Jackson Laboratory