Pitx3

Functionⁱ

Transcriptional regulator which is important for the differentiation and maintenance of meso-diencephalic dopaminergic (mdDA) neurons during development. In addition to its importance during development, it also has roles in the long-term survival and maintenance of the mdDA neurons. Activates NR4A2/NURR1-mediated transcription of genes such as SLC6A3, SLC18A2, TH and DRD2 which are essential for development of mdDA neurons. Acts by decreasing the interaction of NR4A2/NURR1 with the corepressor NCOR2/SMRT which acts through histone deacetylases (HDACs) to keep promoters of NR4A2/NURR1 target genes in a repressed deacetylated state. Essential for the normal lens development and differentiation. Plays a critical role in the maintenance of mitotic activity of lens epithelial cells, fiber cell differentiation and in the control of the temporal and spatial activation of fiber cell-specific crystallins. Positively regulates FOXE3 expression and negatively regulates PROX1 in the anterior lens epithelium, preventing activation of CDKN1B/P27Kip1 and CDKN1C/P57Kip2 and thus maintains lens epithelial cells in cell cycle.5 Publications

Regions

Feature key	Position(s)	DescriptionActions	Graphical view	Length
DNA bindingⁱ	62 – 121	HomeoboxPROSITE-ProRule annotationAdd BLAST		60

GO - Molecular functionⁱ

DNA-binding transcription factor activity Source: MGI
RNA polymerase II proximal promoter sequence-specific DNA binding
Source: NTNU_SB
Inferred from direct assayⁱ
- 11299318
transcriptional activator activity, RNA polymerase II proximal promoter sequence-specific DNA binding
Source: NTNU_SB
Inferred from direct assayⁱ
- 11299318

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

aging Source: Ensembl
cellular response to glial cell derived neurotrophic factor Source: Ensembl
dopaminergic neuron differentiation
Source: UniProtKB
Inferred from direct assayⁱ
- 19144721
lens development in camera-type eye
Source: UniProtKB
Inferred from mutant phenotypeⁱ
- 19007884
- 19334279
lens fiber cell differentiation
Source: UniProtKB
Inferred from mutant phenotypeⁱ
- 19007884
lens morphogenesis in camera-type eye
Source: UniProtKB
Inferred from mutant phenotypeⁱ
- 19334279
locomotory behavior
Source: MGI
Inferred from mutant phenotypeⁱ
- 17919745
midbrain development
Source: UniProtKB
Inferred from mutant phenotypeⁱ
- 15950611
negative regulation of gliogenesis Source: MGI
negative regulation of neurogenesis Source: MGI
neuron development
Source: MGI
Inferred from mutant phenotypeⁱ
- 14973278
positive regulation of cell proliferation in midbrain Source: MGI
positive regulation of neuron apoptotic process Source: MGI
positive regulation of transcription, DNA-templated
Source: UniProtKB
Inferred from direct assayⁱ
- 19144721
positive regulation of transcription by RNA polymerase II
Source: NTNU_SB
Inferred from direct assayⁱ
- 11299318
regulation of gene expression
Source: MGI
Inferred from direct assayⁱ
- 17761882
regulation of transcription, DNA-templated
Source: UniProtKB
Inferred from direct assayⁱ
- 17184956
response to cocaine Source: Ensembl
response to immobilization stress Source: Ensembl
response to methamphetamine hydrochloride Source: Ensembl
response to morphine Source: Ensembl

View the complete GO annotation on QuickGO ...

Keywordsⁱ

Molecular function	Activator, Developmental protein, DNA-binding
Biological process	Transcription, Transcription regulation

Names & Taxonomyⁱ

Protein namesⁱ	Recommended name: Pituitary homeobox 3 Alternative name(s): Homeobox protein PITX3 Paired-like homeodomain transcription factor 3
Gene namesⁱ	Name:Pitx3
Organismⁱ	Mus musculus (Mouse)
Taxonomic identifierⁱ	10090 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Glires › Rodentia › Myomorpha › Muroidea › Muridae › Murinae › Mus › Mus
Proteomesⁱ	UP000000589 Componentⁱ: Chromosome 19

Organism-specific databases

MGIⁱ	MGI:1100498 Pitx3

Keywords - Cellular componentⁱ

Nucleus

Pathology & Biotechⁱ

Involvement in diseaseⁱ

Mutations in Pitx3 appear to be the cause of the aphakia (ak) phenotype, a recessive homozygous disease characterized by small eyes and closed eyelids.1 Publication

Disruption phenotypeⁱ

Mice show loss of nascent substantia nigra dopaminergic neurons at the beginning of their final differentiation and a loss of tyrosine hydroxylase (TH) expression specifically in the substantia nigra neurons.1 Publication

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
ChainⁱPRO_0000049230	1 – 302	Pituitary homeobox 3Add BLAST		302

Amino acid modifications

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Modified residueⁱ	52	PhosphoserineBy similarity		1
Modified residueⁱ	57	PhosphoserineBy similarity		1

Keywords - PTMⁱ

Phosphoprotein

Proteomic databases

PaxDbⁱ	O35160
PRIDEⁱ	O35160

PTM databases

PhosphoSitePlusⁱ	O35160

PhosphoSitePlusⁱ

O35160

Expressionⁱ

Tissue specificityⁱ

Highly expressed in developing eye lens. Expression is restricted to the substantia nigra and ventral tegmental area in the midbrain.2 Publications

Developmental stageⁱ

First visible in E10.5 embryos where expression is confined to the lens vesicles. Between E11.5 and E12.5, expressed in both the lens epithelium and differentiating primary fiber cells. In the late fetal stage after the lens is formed, primarily found in the lens epithelium and the lens equator region where lens epithelial cells exit from the cell cycle and differentiate into fiber cells (at protein level). First expressed in the eye at 10 dpc embryos. Throughout eye development, expressed in the lens placode and forming lens pit. From 12 dpc, also detected in the midbrain region, tongue, incisor primordia, condensing mesenchyme around the sternum and vertebrae and in the head muscles.2 Publications

Gene expression databases

Bgeeⁱ	ENSMUSG00000025229 Expressed in 49 organ(s), highest expression level in lens of camera-type eye
CleanExⁱ	MM_PITX3
ExpressionAtlasⁱ	O35160 baseline and differential
Genevisibleⁱ	O35160 MM

Interactionⁱ

Subunit structureⁱ

Interacts with SFPQ.1 Publication

Protein-protein interaction databases

BioGridⁱ	202188, 1 interactor
STRINGⁱ	10090.ENSMUSP00000026259

Structureⁱ

3D structure databases

ProteinModelPortalⁱ	O35160
SMRⁱ	O35160
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Family & Domainsⁱ

Motif

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Motifⁱ	262 – 275	OARPROSITE-ProRule annotationAdd BLAST		14
Motifⁱ	268 – 272	Nuclear localization signalSequence analysis		5

Compositional bias

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Compositional biasⁱ	240 – 250	Poly-AlaAdd BLAST		11

Sequence similaritiesⁱ

Belongs to the paired homeobox family. Bicoid subfamily.Curated

Keywords - Domainⁱ

Homeobox

Phylogenomic databases

eggNOGⁱ	KOG0486 Eukaryota ENOG410ZZ6J LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00920000148948
HOGENOMⁱ	HOG000273886
HOVERGENⁱ	HBG068770
InParanoidⁱ	O35160
KEGG Orthology (KO) More...KOⁱ	K09357
OMAⁱ	LCKGGFA
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G0H7U
PhylomeDBⁱ	O35160
TreeFamⁱ	TF351940

Family and domain databases

Conserved Domains Database More...CDDⁱ	cd00086 homeodomain, 1 hit
InterProⁱ	View protein in InterPro IPR009057 Homeobox-like_sf IPR017970 Homeobox_CS IPR001356 Homeobox_dom IPR016233 Homeobox_Pitx/unc30 IPR003654 OAR_dom
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF00046 Homeobox, 1 hit PF03826 OAR, 1 hit
PIRSFⁱ	PIRSF000563 Homeobox_protein_Pitx/Unc30, 1 hit
SMARTⁱ	View protein in SMART SM00389 HOX, 1 hit
SUPFAMⁱ	SSF46689 SSF46689, 1 hit
PROSITEⁱ	View protein in PROSITE PS00027 HOMEOBOX_1, 1 hit PS50071 HOMEOBOX_2, 1 hit PS50803 OAR, 1 hit

Sequence (1+)ⁱ

Sequence statusⁱ: Complete.

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show all Align All

O35160-1 [UniParc]FASTA Add to basket

« Hide

        10         20         30         40         50
MEFGLLGEAE ARSPALSLSD AGTPHPPLPE HGCKGQEHSD SEKASASLPG 
        60         70         80         90        100
GSPEDGSLKK KQRRQRTHFT SQQLQELEAT FQRNRYPDMS TREEIAVWTN 
       110        120        130        140        150
LTEARVRVWF KNRRAKWRKR ERSQQAELCK GGFAAPLGGL VPPYEEVYPG 
       160        170        180        190        200
YSYGNWPPKA LAPPLAAKTF PFAFNSVNVG PLASQPVFSP PSSIAASMVP 
       210        220        230        240        250
SAAAAPGTVP GPGALQGLGG APPGLAPAAV SSGAVSCPYA SAAAAAAAAA 
       260        270        280        290        300
SSPYVYRDPC NSSLASLRLK AKQHASFSYP AVPGPPPAAN LSPCQYAVER 

PV

Length:302

Mass (Da):31,715

Last modified:January 1, 1998 - v1

Checksum:ⁱEB6EF6863B349264

GO

Computationally mapped potential isoform sequencesⁱ

There is 1 potential isoform mapped to this entry.BLAST Align Show all Add to basket

	Entry	Entry name		Protein names	Gene names	Length	Annotation

	G3UZN3	G3UZN3_MOUSE		Pituitary homeobox 3 Pituitary homeobox 3	Pitx3	90	Annotation score:

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	AF005772 mRNA Translation: AAB87380.1 BC120844 mRNA Translation: AAI20845.1 BC137810 mRNA Translation: AAI37811.1
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS29872.1
NCBI Reference Sequences More...RefSeqⁱ	NP_032878.1, NM_008852.4
UniGeneⁱ	Mm.6255

Genome annotation databases

Ensemblⁱ	ENSMUST00000026259; ENSMUSP00000026259; ENSMUSG00000025229
GeneIDⁱ	18742
KEGGⁱ	mmu:18742
UCSC genome browser More...UCSCⁱ	uc008hsl.1 mouse

Similar proteinsⁱ

100% Identity
90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length	Source
O35160	Pituitary homeobox 3 (Fragment)		MOUSE		90	UniRef100_O35160
Paired-like homeodomain transcription factor 3		MUSMM		147
UPI0003D75EAF		MOUSE		388

Protein	Similar proteins		Species	Score	Length	Source
O35160	Pituitary homeobox 3 (Fragment)		MOUSE		90	UniRef90_O35160
Paired-like homeodomain transcription factor 3		MUSMM		147
UPI0003D75EAF		MOUSE		388

Protein	Similar proteins		Species	Score	Length	Source
O35160	Isoform Ptx2A of Pituitary homeobox 2		MOUSE		271	UniRef50_P97474-3
Pituitary homeobox 3		XENLA		292
Pituitary homeobox 3		HUMAN		302
Pituitary homeobox 3		RAT		302
Pituitary homeobox 2		DANRE		314
+513

Cross-referencesⁱ

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	AF005772 mRNA Translation: AAB87380.1 BC120844 mRNA Translation: AAI20845.1 BC137810 mRNA Translation: AAI37811.1
CCDSⁱ	CCDS29872.1
RefSeqⁱ	NP_032878.1, NM_008852.4
UniGeneⁱ	Mm.6255

3D structure databases

ProteinModelPortalⁱ	O35160
SMRⁱ	O35160
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Protein-protein interaction databases

BioGridⁱ	202188, 1 interactor
STRINGⁱ	10090.ENSMUSP00000026259

PTM databases

PhosphoSitePlusⁱ	O35160

PhosphoSitePlusⁱ

O35160

Proteomic databases

PaxDbⁱ	O35160
PRIDEⁱ	O35160

Protocols and materials databases

Structural Biology Knowledgebase	Search...

Structural Biology Knowledgebase

Search...

Genome annotation databases

Ensemblⁱ	ENSMUST00000026259; ENSMUSP00000026259; ENSMUSG00000025229
GeneIDⁱ	18742
KEGGⁱ	mmu:18742
UCSCⁱ	uc008hsl.1 mouse

Organism-specific databases

CTDⁱ	5309
MGIⁱ	MGI:1100498 Pitx3

Phylogenomic databases

eggNOGⁱ	KOG0486 Eukaryota ENOG410ZZ6J LUCA
GeneTreeⁱ	ENSGT00920000148948
HOGENOMⁱ	HOG000273886
HOVERGENⁱ	HBG068770
InParanoidⁱ	O35160
KOⁱ	K09357
OMAⁱ	LCKGGFA
OrthoDBⁱ	EOG091G0H7U
PhylomeDBⁱ	O35160
TreeFamⁱ	TF351940

Miscellaneous databases

Protein Ontology More...PROⁱ	PR:O35160
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSMUSG00000025229 Expressed in 49 organ(s), highest expression level in lens of camera-type eye
CleanExⁱ	MM_PITX3
ExpressionAtlasⁱ	O35160 baseline and differential
Genevisibleⁱ	O35160 MM

Family and domain databases

CDDⁱ	cd00086 homeodomain, 1 hit
InterProⁱ	View protein in InterPro IPR009057 Homeobox-like_sf IPR017970 Homeobox_CS IPR001356 Homeobox_dom IPR016233 Homeobox_Pitx/unc30 IPR003654 OAR_dom
Pfamⁱ	View protein in Pfam PF00046 Homeobox, 1 hit PF03826 OAR, 1 hit
PIRSFⁱ	PIRSF000563 Homeobox_protein_Pitx/Unc30, 1 hit
SMARTⁱ	View protein in SMART SM00389 HOX, 1 hit
SUPFAMⁱ	SSF46689 SSF46689, 1 hit
PROSITEⁱ	View protein in PROSITE PS00027 HOMEOBOX_1, 1 hit PS50071 HOMEOBOX_2, 1 hit PS50803 OAR, 1 hit
ProtoNetⁱ	Search...

Entry informationⁱ

Entry nameⁱ	PITX3_MOUSE
Accessionⁱ	O35160Primary (citable) accession number: O35160 Secondary accession number(s): Q0VB03
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	July 15, 1998
	Last sequence update:	January 1, 1998
	Last modified:	September 12, 2018
	This is version 140 of the entry and version 1 of the sequence. See complete history.
Entry statusⁱ	Reviewed (UniProtKB/Swiss-Prot)
Annotation program	Chordata Protein Annotation Program

Miscellaneousⁱ

Keywords - Technical termⁱ

Complete proteome, Reference proteome

Documents

SIMILARITY comments
Index of protein domains and families
MGD cross-references
Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot

UniProtKB

UniParc

Help

UniRef

Proteomes

Annotation systems

Supporting data

UniProtKB - O35160 (PITX3_MOUSE)

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Pituitary homeobox 3

Select a section on the left to see content.

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Regions

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

Organism-specific databases

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Nucleus

Nucleus

Other locations

Keywords - Cellular componenti

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Amino acid modifications

Keywords - PTMi

Proteomic databases

PTM databases

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

Protein-protein interaction databases

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Motif

Compositional bias

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Keywords - Domaini

Phylogenomic databases

Family and domain databases

Sequence databases

Genome annotation databases

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

Sequence databases

3D structure databases

Protein-protein interaction databases

PTM databases

Proteomic databases

Protocols and materials databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Miscellaneous databases

Gene expression databases

Family and domain databases

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

Documents

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Functionⁱ

GO - Molecular functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Keywords - Cellular componentⁱ

Pathology & Biotechⁱ

PTM / Processingⁱ

Keywords - PTMⁱ

Expressionⁱ

Interactionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Keywords - Domainⁱ

Cross-referencesⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ