PITX2
paired like homeodomain 2 | ARP1, Brx1, IGDS, Otlx2, RS, IHG2, IRID2, RGS, RIEG, RIEG1

This gene encodes a member of the RIEG/PITX homeobox family, which is in the bicoid class of homeodomain proteins. The encoded protein acts as a transcription factor and regulates procollagen lysyl hydroxylase gene expression. This protein plays a role in the terminal differentiation of somatotroph and lactotroph cell phenotypes, is involved in the development of the eye, tooth and abdominal organs, and acts as a transcriptional regulator involved in basal and hormone-regulated activity of prolactin. Mutations in this gene are associated with Axenfeld-Rieger syndrome, iridogoniodysgenesis syndrome, and sporadic cases of Peters anomaly. A similar protein in other vertebrates is involved in the determination of left-right asymmetry during development. Alternatively spliced transcript variants encoding distinct isoforms have been described. [provided by RefSeq, Jul 2008]

Developmental clusters: GC2
Biological processes 61 terms
DNA binding (GO:0003677)DNA-binding transcription factor activity (GO:0003700)DNA-binding transcription factor activity (GO:0003700)DNA-binding transcription factor activity, RNA polymerase II-specific (GO:0000981)DNA-binding transcription factor activity, RNA polymerase II-specific (GO:0000981)DNA-binding transcription factor activity, RNA polymerase II-specific (GO:0000981)DNA-binding transcription factor activity, RNA polymerase II-specific (GO:0000981)DNA-binding transcription factor binding (GO:0140297)DNA-binding transcription repressor activity, RNA polymerase II-specific (GO:0001227)RNA polymerase II cis-regulatory region sequence-specific DNA binding (GO:0000978)RNA polymerase II-specific DNA-binding transcription factor binding (GO:0061629)anatomical structure morphogenesis (GO:0009653)animal organ morphogenesis (GO:0009887)camera-type eye development (GO:0043010)cardiac neural crest cell migration involved in outflow tract morphogenesis (GO:0003253)cardiac neural crest cell migration involved in outflow tract morphogenesis (GO:0003253)chromatin (GO:0000785)chromatin DNA binding (GO:0031490)chromatin binding (GO:0003682)cytoplasm (GO:0005737)deltoid tuberosity development (GO:0035993)deltoid tuberosity development (GO:0035993)determination of left/right symmetry (GO:0007368)embryonic heart tube left/right pattern formation (GO:0060971)embryonic heart tube left/right pattern formation (GO:0060971)hair cell differentiation (GO:0035315)identical protein binding (GO:0042802)iris morphogenesis (GO:0061072)left/right axis specification (GO:0070986)left/right axis specification (GO:0070986)negative regulation of transcription by RNA polymerase II (GO:0000122)negative regulation of transcription by RNA polymerase II (GO:0000122)nucleoplasm (GO:0005654)nucleoplasm (GO:0005654)nucleus (GO:0005634)nucleus (GO:0005634)nucleus (GO:0005634)odontogenesis (GO:0042476)outflow tract morphogenesis (GO:0003151)outflow tract morphogenesis (GO:0003151)phosphoprotein binding (GO:0051219)positive regulation of cell population proliferation (GO:0008284)positive regulation of cell population proliferation (GO:0008284)positive regulation of transcription by RNA polymerase II (GO:0045944)prolactin secreting cell differentiation (GO:0060127)protein binding (GO:0005515)protein homodimerization activity (GO:0042803)regulation of DNA-templated transcription (GO:0006355)regulation of DNA-templated transcription (GO:0006355)regulation of transcription by RNA polymerase II (GO:0006357)regulation of transcription by RNA polymerase II (GO:0006357)ribonucleoprotein complex binding (GO:0043021)sequence-specific DNA binding (GO:0043565)sequence-specific double-stranded DNA binding (GO:1990837)somatotropin secreting cell differentiation (GO:0060126)spleen development (GO:0048536)spleen development (GO:0048536)system development (GO:0048731)transcription cis-regulatory region binding (GO:0000976)transcription regulator complex (GO:0005667)transcription regulator complex (GO:0005667)
Expression (TPM)
PITX2 — as a Regulated Gene

TFs regulating PITX2 0 TFs

Transcription factors with Perturb-seq knockdown data for PITX2. The Binding column indicates whether any binding evidence exists for this TF–gene pair (ChIP-seq or motif footprint peaks). The Mean coef is the average Perturb-seq regression coefficient across active gRNAs (positive = PITX2 upregulated upon KD; negative = downregulated). The Outlier column indicates whether this gene is in the top or bottom 5% of all TF knockdown effects.

Data: Effect:
TF Mean coef Binding Outlier TF→Gene link

Elements linked to PITX2

Open chromatin peaks (ATAC-seq) in the genomic neighbourhood of PITX2, linked by TSS proximity or chromatin conformation (Multiome / HiCAR). Each element overlaps at least one TF ChIP-seq binding site — the TFs column shows how many distinct TFs bind that element.

Accessibility Element Dist. to TSS Link type TFs
chr4:110,411,354–110,412,301 207.0 kb Distal (>10kb) Multiome 372
chr4:110,609,129–110,609,424 9.4 kb Proximal (<10kb) 111
chr4:110,613,796–110,614,882 4.5 kb Proximal (<10kb) Multiome 225
chr4:110,615,006–110,616,645 3.0 kb Proximal (<10kb) Multiome 226
chr4:110,617,892–110,620,263 824 bp At TSS Multiome 335
chr4:110,620,638–110,627,477 4.3 kb Proximal (<10kb) Multiome 562
chr4:110,628,237–110,630,191 9.4 kb Proximal (<10kb) 196
chr4:110,630,610–110,633,369 12.0 kb Distal (>10kb) Multiome 525
chr4:110,633,694–110,634,390 4.4 kb Proximal (<10kb) 85
chr4:110,636,247–110,638,157 7.0 kb Proximal (<10kb) 428
chr4:110,639,185–110,639,765 9.9 kb Proximal (<10kb) 111
chr4:110,641,211–110,641,759 8.7 kb Proximal (<10kb) 68
chr4:110,793,382–110,794,932 175.6 kb Distal (>10kb) Multiome 287
chr4:111,795,408–111,796,556 1177.1 kb Distal (>10kb) Multiome HiCAR 207

Genome Browser

Genomic view of the PITX2 locus showing ATAC-seq accessibility and RNA-seq expression across the ESC → DE time course, together with TF binding peaks and element-to-TSS loop connections.

chr4:110,401,354 – 111,806,556
Proximal 1 kb Distal 10 kb Multiome HiCAR ATAC-seq RNA-seq