PRRX1

Paired related homeobox 1 is a protein that in humans is encoded by the PRRX1 gene.[5][6]

PRRX1
Identifiers
Aliases	PRRX1, AGOTC, PHOX1, PMX1, PRX-1, PRX1, paired related homeobox 1
External IDs	OMIM: 167420 MGI: 97712 HomoloGene: 7896 GeneCards: PRRX1
Gene location (Human) Chr. Chromosome 1 (human)[1] Band 1q24.2 Start 170,662,728 bp[1] End 170,739,421 bp[1]
Gene location (Mouse) Chr. Chromosome 1 (mouse)[2] Band 1 H2.1|1 70.53 cM Start 163,245,119 bp[2] End 163,313,710 bp[2]
RNA expression pattern More reference expression data
Gene ontology Molecular function • sequence-specific DNA binding • GO:0001105 transcription coactivator activity • DNA binding • HMG box domain binding • GO:0000980 RNA polymerase II cis-regulatory region sequence-specific DNA binding • GO:0001078, GO:0001214, GO:0001206 DNA-binding transcription repressor activity, RNA polymerase II-specific • GO:0001200, GO:0001133, GO:0001201 DNA-binding transcription factor activity, RNA polymerase II-specific Cellular component • cell nucleus • nucleoplasm • cytosol Biological process • embryonic skeletal system morphogenesis • palate development • regulation of transcription, DNA-templated • neuron fate determination • positive regulation of smoothened signaling pathway • regulation of neuron projection regeneration • negative regulation of transcription from RNA polymerase II promoter • neuronal stem cell population maintenance • multicellular organism development • cartilage development • positive regulation of mesenchymal cell proliferation • embryonic limb morphogenesis • inner ear morphogenesis • artery morphogenesis • middle ear morphogenesis • embryonic cranial skeleton morphogenesis • positive regulation of transcription from RNA polymerase II promoter • transcription from RNA polymerase II promoter Sources:Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	5396	18933
Ensembl	ENSG00000116132	ENSMUSG00000026586
UniProt	P54821	P63013
RefSeq (mRNA)	NM_006902 NM_022716	NM_001025570 NM_011127 NM_175686
RefSeq (protein)	NP_008833 NP_073207	NP_001020741 NP_035257 NP_783617
Location (UCSC)	Chr 1: 170.66 – 170.74 Mb	Chr 1: 163.25 – 163.31 Mb
PubMed search	[3]	[4]
Wikidata
View/Edit Human View/Edit Mouse

Function

The DNA-associated protein encoded by this gene is a member of the paired family of homeobox proteins localized to the nucleus. The protein functions as a transcription coactivator, enhancing the DNA-binding activity of serum response factor, a protein required for the induction of genes by growth and differentiation factors. The protein regulates muscle creatine kinase, indicating a role in the establishment of diverse mesodermal muscle types. Alternative splicing yields two isoforms that differ in abundance and expression patterns.[6]

Role in mesenchymal stem cell differentiation

Prrx1 expression is restricted to the mesoderm during embryonic development, and both Prrx1 and Prrx2 are expressed in mesenchymal tissues in adult mice.[7][8][9][10][11] Mice that lack both Prrx1 and Prrx2 have profound defects in mesenchymal cell differentiation in the craniofacial region.[9][12] Several recent studies demonstrate that PRRX1 can regulate differentiation of mesenchymal precursors. For example, PRRX1 inhibits adipogenesis by activating transforming growth factor-beta (TGF-beta) signaling,[13] and also acts downstream of tumor necrosis factor-alpha to inhibit osteoblast differentiation.[14]

References

1. GRCh38: Ensembl release 89: ENSG00000116132 - Ensembl, May 2017
2. GRCm38: Ensembl release 89: ENSMUSG00000026586 - Ensembl, May 2017
3. "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
4. "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
5. Grueneberg DA, Natesan S, Alexandre C, Gilman MZ (Sep 1992). "Human and Drosophila homeodomain proteins that enhance the DNA-binding activity of serum response factor". Science. 257 (5073): 1089–95. doi:10.1126/science.257.5073.1089. PMID 1509260. S2CID 1206059.
6. "Entrez Gene: PRRX1 paired related homeobox 1".
7. Kern MJ, Witte DP, Valerius MT, Aronow BJ, Potter SS (October 1992). "A novel murine homeobox gene isolated by a tissue specific PCR cloning strategy". Nucleic Acids Res. 20 (19): 5189–95. doi:10.1093/nar/20.19.5189. PMC 334304. PMID 1383943.
8. Nohno T, Koyama E, Myokai F, Taniguchi S, Ohuchi H, Saito T, Noji S (July 1993). "A chicken homeobox gene related to Drosophila paired is predominantly expressed in the developing limb". Dev. Biol. 158 (1): 254–64. doi:10.1006/dbio.1993.1184. PMID 8101172.
9. Lu MF, Cheng HT, Kern MJ, Potter SS, Tran B, Diekwisch TG, Martin JF (February 1999). "prx-1 functions cooperatively with another paired-related homeobox gene, prx-2, to maintain cell fates within the craniofacial mesenchyme" (PDF). Development. 126 (3): 495–504. PMID 9876178.
10. Hu YS, Zhou H, Kartsogiannis V, Eisman JA, Martin TJ, Ng KW (November 1998). "Expression of rat homeobox gene, rHOX, in developing and adult tissues in mice and regulation of its mRNA expression in osteoblasts by bone morphogenetic protein 2 and parathyroid hormone-related protein". Mol. Endocrinol. 12 (11): 1721–32. doi:10.1210/me.12.11.1721. PMID 9817598.
11. Cserjesi P, Lilly B, Bryson L, Wang Y, Sassoon DA, Olson EN (August 1992). "MHox: a mesodermally restricted homeodomain protein that binds an essential site in the muscle creatine kinase enhancer". Development. 115 (4): 1087–101. PMID 1360403.
12. ten Berge D, Brouwer A, Korving J, Martin JF, Meijlink F (October 1998). "Prx1 and Prx2 in skeletogenesis: roles in the craniofacial region, inner ear and limbs". Development. 125 (19): 3831–42. PMID 9729491.
13. Du B, Cawthorn WP, Su A, Doucette CR, Yao Y, Hemati N, Kampert S, McCoin C, Broome DT, Rosen CJ, Yang G, MacDougald OA (February 2013). "The transcription factor paired-related homeobox 1 (Prrx1) inhibits adipogenesis by activating transforming growth factor-β (TGFβ) signaling". J. Biol. Chem. 288 (5): 3036–47. doi:10.1074/jbc.M112.440370. PMC 3561528. PMID 23250756.
14. Lu X, Beck GR, Gilbert LC, Camalier CE, Bateman NW, Hood BL, Conrads TP, Kern MJ, You S, Chen H, Nanes MS (January 2011). "Identification of the homeobox protein Prx1 (MHox, Prrx-1) as a regulator of osterix expression and mediator of tumor necrosis factor α action in osteoblast differentiation". J. Bone Miner. Res. 26 (1): 209–19. doi:10.1002/jbmr.203. PMC 3179318. PMID 20683885.

Further reading

• Grueneberg DA, Simon KJ, Brennan K, Gilman M (1995). "Sequence-specific targeting of nuclear signal transduction pathways by homeodomain proteins". Mol. Cell. Biol. 15 (6): 3318–26. doi:10.1128/MCB.15.6.3318. PMC 230565. PMID 7760827.
• Maruyama K, Sugano S (1994). "Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides". Gene. 138 (1–2): 171–4. doi:10.1016/0378-1119(94)90802-8. PMID 8125298.
• Grueneberg DA, Henry RW, Brauer A, Novina CD, Cheriyath V, Roy AL, Gilman M (October 1997). "A multifunctional DNA-binding protein that promotes the formation of serum response factor/homeodomain complexes: identity to TFII-I". Genes Dev. 11 (19): 2482–93. doi:10.1101/gad.11.19.2482. PMC 316568. PMID 9334314.
• Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, Suyama A, Sugano S (October 1997). "Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library". Gene. 200 (1–2): 149–56. doi:10.1016/S0378-1119(97)00411-3. PMID 9373149.
• Nakamura T, Yamazaki Y, Hatano Y, Miura I (1999). "NUP98 is fused to PMX1 homeobox gene in human acute myelogenous leukemia with chromosome translocation t(1;11)(q23;p15)". Blood. 94 (2): 741–7. doi:10.1182/blood.V94.2.741. PMID 10397741.
• Kataoka K, Yoshitomo-Nakagawa K, Shioda S, Nishizawa M (2001). "A set of Hox proteins interact with the Maf oncoprotein to inhibit its DNA binding, transactivation, and transforming activities". J. Biol. Chem. 276 (1): 819–26. doi:10.1074/jbc.M007643200. PMID 11036080.
• Norris RA, Scott KK, Moore CS, Stetten G, Brown CR, Jabs EW, Wulfsberg EA, Yu J, Kern MJ (November 2000). "Human PRRX1 and PRRX2 genes: cloning, expression, genomic localization, and exclusion as disease genes for Nager syndrome". Mamm. Genome. 11 (11): 1000–5. doi:10.1007/s003350010193. PMID 11063257. S2CID 9937434.
• Jones FS, Meech R, Edelman DB, Oakey RJ, Jones PL (July 2001). "Prx1 controls vascular smooth muscle cell proliferation and tenascin-C expression and is upregulated with Prx2 in pulmonary vascular disease". Circ. Res. 89 (2): 131–8. doi:10.1161/hh1401.093582. PMID 11463719.
• Izmailova E, Bertley FM, Huang Q, Makori N, Miller CJ, Young RA, Aldovini A (February 2003). "HIV-1 Tat reprograms immature dendritic cells to express chemoattractants for activated T cells and macrophages". Nat. Med. 9 (2): 191–7. doi:10.1038/nm822. PMID 12539042. S2CID 26145639.
• Wissmüller S, Kosian T, Wolf M, Finzsch M, Wegner M (2006). "The high-mobility-group domain of Sox proteins interacts with DNA-binding domains of many transcription factors". Nucleic Acids Res. 34 (6): 1735–44. doi:10.1093/nar/gkl105. PMC 1421504. PMID 16582099.
• Gregory SG, Barlow KF, McLay KE, Kaul R, Swarbreck D, Dunham A, Scott CE, Howe KL, Woodfine K, Spencer CC, et al. (May 2006). "The DNA sequence and biological annotation of human chromosome 1". Nature. 441 (7091): 315–21. doi:10.1038/nature04727. PMID 16710414.
• Kim YJ, Ahn JY, Liang P, Ip C, Zhang Y, Park YM (January 2007). "Human prx1 gene is a target of Nrf2 and is up-regulated by hypoxia/reoxygenation: implication to tumor biology". Cancer Res. 67 (2): 546–54. doi:10.1158/0008-5472.CAN-06-2401. PMID 17234762.

External links

• PRRX1+protein,+human at the US National Library of Medicine Medical Subject Headings (MeSH)

This article incorporates text from the United States National Library of Medicine, which is in the public domain.