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Gene Review

Prrx1  -  paired related homeobox 1

Mus musculus

Synonyms: A230024N07Rik, AA755424, AI385634, AI843499, Homeobox protein K-2, ...
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Disease relevance of Prrx1


High impact information on Prrx1


Biological context of Prrx1


Anatomical context of Prrx1

  • Our data reveal that the prx1 limb enhancer is proximally located within the prx1 gene and suggest that prx1 may have an autoregulatory function in limb mesenchyme [1].
  • Mice that lack both Prx1 and Prx2 functions display reduction or absence of skeletal elements in the skull, face, limbs and vertebral column [6].
  • This analysis showed that a conserved domain (PRX), found in both Prx1 and Prx2, activated transcription in NIH 3T3 cells [10].
  • However, the Prx1-/- and Prx1/Prx2 double-null mutants showed a vascular abnormality with an abnormal positioning and awkward curvature of the aortic arch in addition to a misdirected and elongated ductus arteriosus, and in two of seven combined mutants, an anomalous retro-oesophageal right subclavian artery [9].
  • Initial studies revealed that Prx1 localizes to differentiating endothelial cells (ECs) within the fetal lung mesenchyme, and later within ECs forming vascular networks [11].

Associations of Prrx1 with chemical compounds

  • Evidence is provided that this increase of fibrin binding is mediated by the kringle 2 (K2) domain that contains a lysine-binding site [12].
  • The k1 value for tcwt-rtPA was not influenced by the presence of epsilon-aminocaproic acid, suggesting that the lysine-binding site associated with the kringle 2 (K2) region of tPA does not modulate the rate of its initial interaction with rPAI-1 [13].
  • This localization strongly suggests that CLC-K2 confers the basolateral conductance in the type A intercalated cells where Cl(-) is taken up by the anion exchanger in exchange for HCO(3)(-) at the basolateral membranes [14].
  • CLC-K2, a kidney-specific member of the CLC chloride channel family, is thought to play an important role in the transepithelial Cl(-) transport in the kidney [14].
  • These results suggest that a novel set of zinc finger proteins may help regulate the strict tissue and nephron segment-specific expression of CLC-K1 and CLC-K2 channel genes through their GA cis element [15].

Other interactions of Prrx1


Analytical, diagnostic and therapeutic context of Prrx1


  1. Identification of a prx1 limb enhancer. Martin, J.F., Olson, E.N. Genesis (2000) [Pubmed]
  2. Nonredundant antioxidant defense by multiple two-cysteine peroxiredoxins in human prostate cancer cells. Shen, C., Nathan, C. Mol. Med. (2002) [Pubmed]
  3. RmpA2, an activator of capsule biosynthesis in Klebsiella pneumoniae CG43, regulates K2 cps gene expression at the transcriptional level. Lai, Y.C., Peng, H.L., Chang, H.Y. J. Bacteriol. (2003) [Pubmed]
  4. FAK induces expression of Prx1 to promote tenascin-C-dependent fibroblast migration. McKean, D.M., Sisbarro, L., Ilic, D., Kaplan-Alburquerque, N., Nemenoff, R., Weiser-Evans, M., Kern, M.J., Jones, P.L. J. Cell Biol. (2003) [Pubmed]
  5. Genetic dissection of Pitx2 in craniofacial development uncovers new functions in branchial arch morphogenesis, late aspects of tooth morphogenesis and cell migration. Liu, W., Selever, J., Lu, M.F., Martin, J.F. Development (2003) [Pubmed]
  6. Prx1 and Prx2 are upstream regulators of sonic hedgehog and control cell proliferation during mandibular arch morphogenesis. ten Berge, D., Brouwer, A., Korving, J., Reijnen, M.J., van Raaij, E.J., Verbeek, F., Gaffield, W., Meijlink, F. Development (2001) [Pubmed]
  7. prx-1 functions cooperatively with another paired-related homeobox gene, prx-2, to maintain cell fates within the craniofacial mesenchyme. Lu, M.F., Cheng, H.T., Kern, M.J., Potter, S.S., Tran, B., Diekwisch, T.G., Martin, J.F. Development (1999) [Pubmed]
  8. Human PRRX1 and PRRX2 genes: cloning, expression, genomic localization, and exclusion as disease genes for Nager syndrome. Norris, R.A., Scott, K.K., Moore, C.S., Stetten, G., Brown, C.R., Jabs, E.W., Wulfsberg, E.A., Yu, J., Kern, M.J. Mamm. Genome (2000) [Pubmed]
  9. Loss of function of the Prx1 and Prx2 homeobox genes alters architecture of the great elastic arteries and ductus arteriosus. Bergwerff, M., Gittenberger-de Groot, A.C., Wisse, L.J., DeRuiter, M.C., Wessels, A., Martin, J.F., Olson, E.N., Kern, M.J. Virchows Arch. (2000) [Pubmed]
  10. Identification of domains mediating transcription activation, repression, and inhibition in the paired-related homeobox protein, Prx2 (S8). Norris, R.A., Kern, M.J. DNA Cell Biol. (2001) [Pubmed]
  11. Paired-related homeobox gene Prx1 is required for pulmonary vascular development. Ihida-Stansbury, K., McKean, D.M., Gebb, S.A., Martin, J.F., Stevens, T., Nemenoff, R., Akeson, A., Vaughn, J., Jones, P.L. Circ. Res. (2004) [Pubmed]
  12. Identification of the domains of tissue-type plasminogen activator involved in the augmented binding to fibrin after limited digestion with plasmin. de Vries, C., Veerman, H., Pannekoek, H. J. Biol. Chem. (1989) [Pubmed]
  13. Structural determinants of the noncatalytic chain of tissue-type plasminogen activator that modulate its association rate with plasminogen activator inhibitor-1. de Serrano, V.S., Castellino, F.J. J. Biol. Chem. (1990) [Pubmed]
  14. Intrarenal and cellular localization of CLC-K2 protein in the mouse kidney. Kobayashi, K., Uchida, S., Mizutani, S., Sasaki, S., Marumo, F. J. Am. Soc. Nephrol. (2001) [Pubmed]
  15. Isolation of a novel zinc finger repressor that regulates the kidney-specific CLC-K1 promoter. Uchida, S., Sasaki, S., Marumo, F. Kidney Int. (2001) [Pubmed]
  16. Genomic organization and chromosome localization of the murine homeobox gene Pmx. Kern, M.J., Argao, E.A., Birkenmeier, E.H., Rowe, L.B., Potter, S.S. Genomics (1994) [Pubmed]
  17. A chicken homeobox gene related to Drosophila paired is predominantly expressed in the developing limb. Nohno, T., Koyama, E., Myokai, F., Taniguchi, S., Ohuchi, H., Saito, T., Noji, S. Dev. Biol. (1993) [Pubmed]
  18. A novel murine homeobox gene isolated by a tissue specific PCR cloning strategy. Kern, M.J., Witte, D.P., Valerius, M.T., Aronow, B.J., Potter, S.S. Nucleic Acids Res. (1992) [Pubmed]
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