Q9QUS4 · HEY2_MOUSE

  • Protein
    Hairy/enhancer-of-split related with YRPW motif protein 2
  • Gene
    Hey2
  • Status
    UniProtKB reviewed (Swiss-Prot)
  • Amino acids
  • Protein existence
    Evidence at protein level
  • Annotation score
    5/5

Function

function

Transcriptional repressor which functions as a downstream effector of Notch signaling in cardiovascular development. Specifically required for the Notch-induced endocardial epithelial to mesenchymal transition, which is itself criticial for cardiac valve and septum development. May be required in conjunction with HEY1 to specify arterial cell fate or identity. Promotes maintenance of neuronal precursor cells and glial versus neuronal fate specification. Binds preferentially to the canonical E box sequence 5'-CACGTG-3'. Represses transcription by the cardiac transcriptional activators GATA4 and GATA6 and by the neuronal bHLH factors ASCL1/MASH1 and NEUROD4/MATH3.

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 Componentcytoplasm
Cellular Componentnucleoplasm
Cellular Componentnucleus
Cellular Componenttranscription regulator complex
Cellular Componenttranscription repressor complex
Molecular Functioncis-regulatory region sequence-specific DNA binding
Molecular FunctionDNA binding
Molecular FunctionDNA-binding transcription factor activity
Molecular FunctionDNA-binding transcription factor activity, RNA polymerase II-specific
Molecular FunctionDNA-binding transcription repressor activity, RNA polymerase II-specific
Molecular Functionhistone deacetylase binding
Molecular Functionidentical protein binding
Molecular Functionprotein dimerization activity
Molecular FunctionRNA polymerase II cis-regulatory region sequence-specific DNA binding
Molecular FunctionRNA polymerase II-specific DNA-binding transcription factor binding
Molecular Functionsequence-specific DNA binding
Biological Processanterior/posterior axis specification
Biological Processanterior/posterior pattern specification
Biological Processaortic valve morphogenesis
Biological Processarterial endothelial cell differentiation
Biological Processartery development
Biological Processascending aorta morphogenesis
Biological Processatrial septum morphogenesis
Biological Processatrioventricular valve development
Biological Processblood vessel development
Biological Processblood vessel endothelial cell differentiation
Biological Processcardiac epithelial to mesenchymal transition
Biological Processcardiac left ventricle morphogenesis
Biological Processcardiac muscle cell apoptotic process
Biological Processcardiac muscle cell proliferation
Biological Processcardiac muscle hypertrophy
Biological Processcardiac muscle hypertrophy in response to stress
Biological Processcardiac right ventricle morphogenesis
Biological Processcardiac septum morphogenesis
Biological Processcardiac vascular smooth muscle cell development
Biological Processcardiac ventricle morphogenesis
Biological Processcell fate commitment
Biological Processcirculatory system development
Biological Processcochlea development
Biological Processcoronary vasculature morphogenesis
Biological Processdorsal aorta morphogenesis
Biological Processendocardial cushion to mesenchymal transition involved in heart valve formation
Biological Processheart trabecula formation
Biological Processlabyrinthine layer blood vessel development
Biological Processmesenchymal cell development
Biological Processmuscular septum morphogenesis
Biological Processnegative regulation of biomineral tissue development
Biological Processnegative regulation of cardiac muscle cell apoptotic process
Biological Processnegative regulation of cardiac vascular smooth muscle cell differentiation
Biological Processnegative regulation of DNA-templated transcription
Biological Processnegative regulation of gene expression
Biological Processnegative regulation of Notch signaling pathway
Biological Processnegative regulation of smooth muscle cell differentiation
Biological Processnegative regulation of transcription by RNA polymerase II
Biological Processnegative regulation of transcription initiation by RNA polymerase II
Biological ProcessNotch signaling pathway
Biological Processoutflow tract morphogenesis
Biological Processpattern specification process
Biological Processpositive regulation of cardiac muscle cell proliferation
Biological Processpositive regulation of heart rate
Biological Processpositive regulation of transcription by RNA polymerase II
Biological Processprotein-DNA complex assembly
Biological Processpulmonary artery morphogenesis
Biological Processpulmonary valve morphogenesis
Biological Processregulation of gene expression
Biological Processregulation of inner ear auditory receptor cell differentiation
Biological Processregulation of neurogenesis
Biological Processregulation of transcription by RNA polymerase II
Biological Processregulation of vasculogenesis
Biological Processtricuspid valve formation
Biological Processtricuspid valve morphogenesis
Biological Processumbilical cord morphogenesis
Biological Processvasculogenesis
Biological Processventricular cardiac muscle cell development
Biological Processventricular septum morphogenesis
Biological Processventricular trabecula myocardium morphogenesis

Keywords

Names & Taxonomy

Protein names

  • Recommended name
    Hairy/enhancer-of-split related with YRPW motif protein 2
  • Alternative names
    • HES-related repressor protein 2
    • Hairy and enhancer of split-related protein 2 (HESR-2)
    • Hairy-related transcription factor 2 (HRT-2; mHRT2)
    • Protein gridlock homolog

Gene names

    • Name
      Hey2
    • Synonyms
      Chf1, Herp, Herp1, Hesr2, Hrt2

Organism names

  • Taxonomic identifier
  • Strain
    • C57BL/6J
  • Taxonomic lineage
    Eukaryota > Metazoa > Chordata > Craniata > Vertebrata > Euteleostomi > Mammalia > Eutheria > Euarchontoglires > Glires > Rodentia > Myomorpha > Muroidea > Muridae > Murinae > Mus > Mus

Accessions

  • Primary accession
    Q9QUS4
  • Secondary accessions
    • Q3TZ99
    • Q8CD44

Proteomes

Organism-specific databases

Subcellular Location

Keywords

Phenotypes & Variants

Disruption phenotype

Mice display a spectrum of cardiac malformations including ventricular septal defects, tetralogy of Fallot and tricuspid atresia. The penetrance of the cardiac malformation phenotype varies according to the strain, suggesting the presence of modifier genes.

Features

Showing features for mutagenesis.

TypeIDPosition(s)Description
Mutagenesis33-37Abrogates interaction with GATA4 and repression of GATA4-mediated transcription.
Mutagenesis49-61Abrogates DNA-binding and transcriptional repression.

Variants

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

The viewer provides 10 variants from UniProt as well as other sources including ClinVar and dbSNP.

Go to variant viewer

PTM/Processing

Features

Showing features for chain.

TypeIDPosition(s)Description
ChainPRO_00002455161-339

Proteomic databases

PTM databases

Expression

Tissue specificity

Highly expressed in the aorta, lower expression detected in the heart, brain, kidney, lung, muscle, ovary and testis.

Induction

By activation of the Notch signaling pathway.

Developmental stage

Expressed in the developing somites and the ventricles of the heart. Expressed in the otic vesicles between 8.5 dpc and 10.5 dpc. Expressed in the myocardium of the ventricles at 9.5 dpc and in the atrioventricular cushions from 9.5 to 12.5 dpc. At 10.5 dpc, strongly expressed in the spinal nerves, the cranial ganglia and the telencephalon. At 11.5 dpc, expressed in the craniofacial region of the distal part of the maxillary arch, along the rostral mandibular arch and surrounding the lateral nasal processes. Expressed in the midbrain-hindbrain boundary and the posterior edge of the hand- and foot-paddle. Expressed in the mediodorsal region of the telencephalon and the ventricular zone of the ventral spinal cord at 12 dpc, then in the ventral region of the telencephalon and the cortical plate at 15 dpc. Expression in the heart is limited to the compact myocardial layer at 17.5 dpc. Also expressed in the developing retina up to P5, at which point expression decreases.

Gene expression databases

Interaction

Subunit

May self-associate (By similarity).
Interacts with ARNT (By similarity).
Interacts with GATA4, GATA6, HES1 and HEYL. Interacts with HDAC1, NCOR1 and SIN3A

Protein-protein interaction databases

Miscellaneous

Structure

Family & Domains

Features

Showing features for region, compositional bias, domain, motif.

TypeIDPosition(s)Description
Region1-52Disordered
Compositional bias24-48Polar residues
Region47-116Transcriptional repression and interaction with NCOR1 and SIN3A
Domain48-103bHLH
Domain122-157Orange
Compositional bias310-331Polar residues
Region310-339Disordered
Motif329-332YQPW motif

Sequence similarities

Belongs to the HEY family.

Phylogenomic databases

Family and domain databases

Sequence

  • Sequence status
    Complete
  • Length
    339
  • Mass (Da)
    35,873
  • Last updated
    2000-05-01 v1
  • Checksum
    105BEEA06DE50444
MKRPCEETTSESDLDETIDVGSENNYPGHATSSVMRSNSPTTTSQIMARKKRRGIIEKRRRDRINNSLSELRRLVPTAFEKQGSAKLEKAEILQMTVDHLKMLQATGGKGYFDAHALATDFMSIGFRECLTEVARYLSSVEGLDPSDPLRVRLVSHLSTCASQREAAVMTSSMAHHHHPLHPHHWAAAFHHLPTALLQPNGLHTSESTPCRLSTSSEVPSAHGSALLTATFAHADSALRMPSGGTVAPCVPPLSTSLLSLSATVHAAAAAATAAAHSFPLSFAGAFPMLPSNAAAAAAVAAATAISPPLSVSAASSPQQTSTGTNNKPYQPWGTEVGAF

Features

Showing features for sequence conflict, compositional bias.

TypeIDPosition(s)Description
Sequence conflict7in Ref. 6; BAC27428
Compositional bias24-48Polar residues
Sequence conflict284in Ref. 6; BAE34310
Compositional bias310-331Polar residues

Keywords

Sequence databases

Nucleotide SequenceProtein SequenceMolecule TypeStatus
AF172287
EMBL· GenBank· DDBJ
AAF14546.1
EMBL· GenBank· DDBJ
mRNA
AJ271867
EMBL· GenBank· DDBJ
CAB71346.1
EMBL· GenBank· DDBJ
mRNA
AF173902
EMBL· GenBank· DDBJ
AAF20174.1
EMBL· GenBank· DDBJ
mRNA
AF232240
EMBL· GenBank· DDBJ
AAF37298.1
EMBL· GenBank· DDBJ
mRNA
AB093589
EMBL· GenBank· DDBJ
BAC55066.1
EMBL· GenBank· DDBJ
mRNA
AK031506
EMBL· GenBank· DDBJ
BAC27428.1
EMBL· GenBank· DDBJ
mRNA
AK158000
EMBL· GenBank· DDBJ
BAE34310.1
EMBL· GenBank· DDBJ
mRNA
BC103575
EMBL· GenBank· DDBJ
AAI03576.1
EMBL· GenBank· DDBJ
mRNA
BC103576
EMBL· GenBank· DDBJ
AAI03577.1
EMBL· GenBank· DDBJ
mRNA

Genome annotation databases

Similar Proteins

Disclaimer

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