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P42581 · HMX3_MOUSE

  • Protein
    Homeobox protein HMX3
  • Gene
    Hmx3
  • Status
    UniProtKB reviewed (Swiss-Prot)
  • Amino acids
  • Protein existence
    Evidence at protein level
  • Annotation score
    5/5

Function

function

Transcription factor involved in specification of neuronal cell types and which is required for inner ear and hypothalamus development. Binds to the 5'-CAAGTG-3' core sequence. Controls semicircular canal formation in the inner ear. Also required for hypothalamic/pituitary axis of the CNS.

Features

Showing features for dna binding.

135650100150200250300350
TypeIDPosition(s)Description
DNA binding227-286Homeobox

GO annotations

all annotationsall molecular functionvirus receptor activitydna bindingrna bindingcytoskeletal motor activitycatalytic activitygtpase activitystructural molecule activitytransporter activitycytoskeletal protein bindinglipid bindingcyclase activityantioxidant activityoxidoreductase activitytransferase activityhydrolase activitylyase activityisomerase activityligase activityprotein tag activitycargo receptor activityhistone bindingprotein folding chaperonetranslation regulator activitynutrient reservoir activityreceptor ligand activitymolecular transducer activitymolecular adaptor activitytoxin activitycell adhesion mediator activitymolecular function regulator activityvirus coreceptor activitycatalytic activity, acting on a proteincatalytic activity, acting on dnacatalytic activity, acting on rnamolecular carrier activitytranscription regulator activitygeneral transcription initiation factor activitymolecular sensor activitymolecular sequestering activityatp-dependent activityother molecular functionall biological processmitotic cell cyclecytokinesiscytoplasmic translationimmune system processmuscle system processcirculatory system processrenal system processrespiratory system processcarbohydrate metabolic processgeneration of precursor metabolites and energydna replicationdna repairdna recombinationchromatin organizationdna-templated transcriptionregulation of dna-templated transcriptiontrna metabolic processprotein foldingprotein glycosylationamino acid metabolic processmodified amino acid metabolic processlipid metabolic processvitamin metabolic processsulfur compound metabolic processintracellular protein transportnucleocytoplasmic transportautophagyinflammatory responsemitochondrion organizationcytoskeleton organizationmicrotubule-based movementperoxisome organizationlysosome organizationchromosome segregationcell adhesionestablishment or maintenance of cell polarityprogrammed cell deathphotosynthesismrna metabolic processsnrna metabolic processvesicle-mediated transportreproductive processdigestive system processsignalingcell differentiationprotein catabolic processextracellular matrix organizationregulatory ncrna-mediated gene silencingtelomere organizationcell junction organizationwound healingribosome biogenesiscilium organizationanatomical structure developmentcell motilitynervous system processendocrine processprotein maturationtransmembrane transportnucleobase-containing small molecule metabolic processhepaticobiliary system processmembrane organizationprotein-containing complex assemblycell wall organization or biogenesisnitrogen cycle metabolic processprotein localization to plasma membranedefense response to other organismdetoxificationmeiotic nuclear divisionmitotic nuclear divisionmitochondrial gene expressioncarbohydrate derivative metabolic processother biological processall cellular componentnuclear chromosomeextracellular regionextracellular spacecell wallnucleusnuclear envelopenucleoplasmchromosomenucleolusmitochondrionlysosomeendosomevacuoleperoxisomeendoplasmic reticulumgolgi apparatuslipid dropletmicrotubule organizing centercytosolribosomecytoskeletonplasma membraneciliumplastidthylakoidexternal encapsulating structureextracellular matrixcytoplasmic vesicleorganelleother cellular component
Cell color indicative of number of GO terms
AspectTerm
Cellular Componentnucleus
Molecular FunctionDNA-binding transcription factor activity, RNA polymerase II-specific
Molecular FunctionRNA polymerase II transcription regulatory region sequence-specific DNA binding
Molecular Functionsequence-specific DNA binding
Biological Processbrain development
Biological Processcell differentiation
Biological Processear development
Biological Processembryo implantation
Biological Processinner ear morphogenesis
Biological Processmaternal process involved in female pregnancy
Biological Processneuromuscular process controlling balance

Keywords

Names & Taxonomy

Protein names

  • Recommended name
    Homeobox protein HMX3
  • Alternative names
    • Homeobox protein H6 family member 3
    • Homeobox protein Nkx-5.1

Gene names

    • Name
      Hmx3
    • Synonyms
      Nkx-5.1, Nkx5-1

Organism names

  • Taxonomic identifier
  • Strain
    • ICR X Swiss Webster
  • Taxonomic lineage
    Eukaryota > Metazoa > Chordata > Craniata > Vertebrata > Euteleostomi > Mammalia > Eutheria > Euarchontoglires > Glires > Rodentia > Myomorpha > Muroidea > Muridae > Murinae > Mus > Mus

Accessions

  • Primary accession
    P42581
  • Secondary accessions
    • B9EIJ0
    • O88699

Proteomes

Organism-specific databases

Subcellular Location

Keywords

Phenotypes & Variants

Disruption phenotype

Mice display abnormal circling behavior and severe vestibular defects owing to a depletion of sensory cells in the saccule and utricle, and a complete loss of the horizontal semicircular canal crista, as well as a fusion of the utricle and saccule endolymphatic spaces into a common utriculosaccular cavity. Moreover, most females have a reproductive defect: females can be fertilized and their embryos undergo normal preimplantation development, but the embryos fail to implant successfully in the uterus and subsequently die. Mice lacking both Hmx2 and Hmx3 show a complete loss of balance, postnatal dwarfism, defects in neuroendocrine system, disturbed hypothalamic-pituitary axis and subsequent die. Defects caused in mice lacking both Hmx2 and Hmx3 can be rescued by expressing the Drosophila Hmx protein.

Features

Showing features for mutagenesis.

TypeIDPosition(s)Description
Mutagenesis276Abolishes DNA-binding.

Variants

We now provide the "Disease & Variants" viewer in its own tab.

The viewer provides 3 variants from UniProt as well as other sources including ClinVar and dbSNP.

Go to variant viewer

PTM/Processing

Features

Showing features for chain, modified residue.

Type
IDPosition(s)Description
ChainPRO_00000489491-356Homeobox protein HMX3
Modified residue153Phosphoserine
Modified residue180Phosphoserine

Keywords

Proteomic databases

PTM databases

Expression

Tissue specificity

Expressed in the developing CNS, including a specific expression in vestibular structures throughout inner ear development.

Induction

Regulated by Six1 and FGFs in inner ear.

Developmental stage

Begins at embryonic day 10.5 in the developing ear, hypothalamus, the neural tube and dorsal root ganglia. It continues to be active throughout prenatal life in discrete regions of the brain with an anterior border in the ventral diencephalon at the optic chiasma and expression domains in mesencephalon, metencephalon, and myelencephalon. At midgestation, it is also expressed in mesenchyme of the head and branchial arches, and in some cranial ganglia, as well as in derivatives of neural crest, such as the truncus sympathicus and myenteric ganglia. In inner ear, it is expressed between 13.5 dpc and birth in non-sensory epithelium of the semicircular canals, utricle and saccule. Also expressed in the cochlea, where the expression is restricted to the stria vascularis.

Gene expression databases

Interaction

Protein-protein interaction databases

Miscellaneous

Structure

Family & Domains

Features

Showing features for compositional bias, region.

Type
IDPosition(s)Description
Compositional bias1-28Pro residues
Region1-57Disordered
Region129-229Disordered
Compositional bias154-190Basic and acidic residues

Sequence similarities

Belongs to the HMX homeobox family.

Keywords

Phylogenomic databases

Family and domain databases

Sequence

  • Sequence status
    Complete
  • Length
    356
  • Mass (Da)
    37,859
  • Last updated
    2011-07-27 v3
  • MD5 Checksum
    8A7B231B77218E6F7F4BBA70895E78F7
MPEPGPDASGTASAPPPQPPPQPPAPKESPFSIRNLLNGDHHRPPPKPQPPPRTLFAPASAAAAAAAAAAAAAKGALEGAAGFALSQVGDLAFPRFEIPAQRFALPAHYLERSPAWWYPYTLTPAGGHLPRPEASEKALLRDSSPASGTDRDSPEPLLKADPDHKELDSKSPDEIILEESDSEEGKKEGEAVPGAAGTTVGATTATPGSEDWKAGAESPEKKPACRKKKTRTVFSRSQVFQLESTFDMKRYLSSSERAGLAASLHLTETQVKIWFQNRRNKWKRQLAAELEAANLSHAAAQRIVRVPILYHENSAAEGAAAAAGAPVPVSQPLLTFPHPVYYSHPVVSSVPLLRPV

Sequence caution

The sequence CAA53079.1 differs from that shown. Reason: Miscellaneous discrepancy Chimeric cDNA.

Features

Showing features for compositional bias, sequence conflict.

Type
IDPosition(s)Description
Compositional bias1-28Pro residues
Sequence conflict68in Ref. 2; CAA08911
Compositional bias154-190Basic and acidic residues

Keywords

Sequence databases

Nucleotide SequenceProtein SequenceMolecule TypeStatus
X75330
EMBL· GenBank· DDBJ
CAA53079.1
EMBL· GenBank· DDBJ
mRNA Sequence problems.
AJ009935
EMBL· GenBank· DDBJ
CAA08911.1
EMBL· GenBank· DDBJ
Genomic DNA
CH466531
EMBL· GenBank· DDBJ
EDL17717.1
EMBL· GenBank· DDBJ
Genomic DNA
BC139482
EMBL· GenBank· DDBJ
AAI39483.1
EMBL· GenBank· DDBJ
mRNA
BC139483
EMBL· GenBank· DDBJ
AAI39484.1
EMBL· GenBank· DDBJ
mRNA

Genome annotation databases

Similar Proteins

Disclaimer

Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. Our staff consists of biologists and biochemists that are not trained to give medical advice.
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