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Gene Review

RPS4X  -  ribosomal protein S4, X-linked

Homo sapiens

Synonyms: 40S ribosomal protein S4, X isoform, CCG2, DXS306, FLJ40595, RPS4, ...
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Disease relevance of RPS4X

  • Two genes with these properties are ZFX and RPS4X, both of which have been proposed to play a role in Turner's syndrome [1].
  • CONCLUSIONS: Scar endometrioma commonly presents as an abdominal mass with noncyclical symptoms [2].
  • Linkage analysis using the polymorphic loci DXS369, DXS296, DXS297 and DXS306 was carried out on a cohort of 17 families segregating for fragile X syndrome [3].
  • BACKGROUND AND OBJECTIVE: Scar sarcoidosis is a circumscribed form of cutaneous sarcoidosis, which is often very difficult to treat [4].
  • In each image, myocardial segments were categorized based on the extent of infarction they contained, with 6 categories each for Percent Scar and Transmurality: normal, from healthy volunteers; and 0%; 1-25%, 26-50%, 51-75%, and > 76% from patients [5].

High impact information on RPS4X

  • Here we show that the human Y- and X-encoded ribosomal proteins, RPS4Y and RPS4X, are interchangeable and provide an essential function: either protein rescued a mutant hamster cell line that was otherwise incapable of growth at modestly elevated temperatures [6].
  • These findings are consistent with the hypothesis that RPS4 deficiency has a role in Turner syndrome, a complex human phenotype associated with monosomy X [6].
  • RPS4X maps to the long arm of the X chromosome (Xq), where no other genes are known to escape X inactivation [7].
  • We consider the possible role of RPS4 haploinsufficiency in the etiology of the Turner phenotype [7].
  • Preincubating Scar and Arp2/3 complex with actin filaments overcomes the initial lag in polymerization, suggesting that efficient nucleation by the Arp2/3 complex requires assembly on the side of a preexisting filament-a dendritic nucleation mechanism [8].

Chemical compound and disease context of RPS4X

  • METHODS: Scar volume was quantified using a validated three-dimensional speckle-pattern stereophotogrammetry before and for a minimum of 8 weeks after intralesional steroid therapy in 12 patients with keloid scars [9].

Biological context of RPS4X


Anatomical context of RPS4X


Associations of RPS4X with chemical compounds

  • A proline-rich domain in the middle of Scar enhances the activity of the W and A domains [8].
  • BACKGROUND: Scar revision with CO2 and Er:YAG lasers has become popular in recent years [18].
  • CONCLUSIONS: Scar in the contralateral kidney seen on DMSA scan seems to predict contralateral reflux after unilateral antireflux surgery [19].

Other interactions of RPS4X


Analytical, diagnostic and therapeutic context of RPS4X

  • Molecular cloning of the human gene, CCG2, that complements the BHK-derived temperature-sensitive cell cycle mutant tsBN63: identity of CCG2 with the human X chromosomal SCAR/RPS4X gene [12].
  • Southern blot analysis showed that four of the seven clones originate from the human genome, three of these contain repeat sequences, and one, SCR10, is devoid of repeats [22].
  • 8. Western blot analysis showed that rapidly proliferating cells overexpress the RPS4 protein in comparison with nonrecognized cell subsets [23].
  • Although the Vancouver Scar Scale has been used as the standard for objective measurements, there are problems with both the validity and reliability of this instrument [24].
  • An occupational therapist, blinded to the perfusion level, rated each scar using the Vancouver Scar Scale [25].


  1. X-chromosome inactivation may explain the difference in viability of XO humans and mice. Ashworth, A., Rastan, S., Lovell-Badge, R., Kay, G. Nature (1991) [Pubmed]
  2. Abdominal wall endometriomas. Blanco, R.G., Parithivel, V.S., Shah, A.K., Gumbs, M.A., Schein, M., Gerst, P.H. Am. J. Surg. (2003) [Pubmed]
  3. Tightly linked polymorphic markers for fragile X syndrome and prenatal cytogenetic diagnostic experience. Glass, I.A., Del Mastro, R.G., Lanyon, W.G., Raeburn, J.A., Kilpatrick, M.W., Webb, T.P., Connor, J.M. Am. J. Med. Genet. (1992) [Pubmed]
  4. Scar sarcoidosis--treatment with the Q-switched ruby laser. Grema, H., Greve, B., Raulin, C. Lasers in surgery and medicine. (2002) [Pubmed]
  5. Relationship between the extent of non-viable myocardium and regional left ventricular function in chronic ischemic heart disease. Kolipaka, A., Chatzimavroudis, G.P., White, R.D., Lieber, M.L., Setser, R.M. Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance. (2005) [Pubmed]
  6. Functional equivalence of human X- and Y-encoded isoforms of ribosomal protein S4 consistent with a role in Turner syndrome. Watanabe, M., Zinn, A.R., Page, D.C., Nishimoto, T. Nat. Genet. (1993) [Pubmed]
  7. Homologous ribosomal protein genes on the human X and Y chromosomes: escape from X inactivation and possible implications for Turner syndrome. Fisher, E.M., Beer-Romero, P., Brown, L.G., Ridley, A., McNeil, J.A., Lawrence, J.B., Willard, H.F., Bieber, F.R., Page, D.C. Cell (1990) [Pubmed]
  8. Scar, a WASp-related protein, activates nucleation of actin filaments by the Arp2/3 complex. Machesky, L.M., Mullins, R.D., Higgs, H.N., Kaiser, D.A., Blanchoin, L., May, R.C., Hall, M.E., Pollard, T.D. Proc. Natl. Acad. Sci. U.S.A. (1999) [Pubmed]
  9. Objective assessment of keloid scars with three-dimensional imaging: quantifying response to intralesional steroid therapy. Ardehali, B., Nouraei, S.A., Van Dam, H., Dex, E., Wood, S., Nduka, C. Plast. Reconstr. Surg. (2007) [Pubmed]
  10. Relationship between the monosomy X phenotype and Y-linked ribosomal protein S4 (Rps4) in several species of mammals: a molecular evolutionary analysis of Rps4 homologs. Omoe, K., Endo, A. Genomics (1996) [Pubmed]
  11. A proposed path by which genes common to mammalian X and Y chromosomes evolve to become X inactivated. Jegalian, K., Page, D.C. Nature (1998) [Pubmed]
  12. Molecular cloning of the human gene, CCG2, that complements the BHK-derived temperature-sensitive cell cycle mutant tsBN63: identity of CCG2 with the human X chromosomal SCAR/RPS4X gene. Watanabe, M., Furuno, N., Goebl, M., Go, M., Miyauchi, K., Sekiguchi, T., Basilico, C., Nishimito, T. J. Cell. Sci. (1991) [Pubmed]
  13. Ullrich-Turner syndrome is not caused by haploinsufficiency of RPS4X. Geerkens, C., Just, W., Held, K.R., Vogel, W. Hum. Genet. (1996) [Pubmed]
  14. Maintenance of X inactivation of the Rps4, Zfx, and Ube1 genes in a mouse in vitro system. Bressler, S.L., Lee, K.H., Adler, D.A., Chapman, V.M., Disteche, C.M. Somat. Cell Mol. Genet. (1993) [Pubmed]
  15. Tree ferns: monophyletic groups and their relationships as revealed by four protein-coding plastid loci. Korall, P., Pryer, K.M., Metzgar, J.S., Schneider, H., Conant, D.S. Mol. Phylogenet. Evol. (2006) [Pubmed]
  16. Genes for the ribosomal S4 protein encoded in higher plant mitochondria are transcribed, edited, and translated. Handa, H., Kubo, N., Kadowaki, K. Mol. Gen. Genet. (1998) [Pubmed]
  17. The right paratracheal stripe in children. Savoca, C.J., Brasch, R.C., Gooding, C.A., Gamsu, G. Pediatric radiology. (1978) [Pubmed]
  18. Treatment of atrophic facial acne scars with a dual-mode Er:YAG laser. Tanzi, E.L., Alster, T.S. Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]. (2002) [Pubmed]
  19. Contralateral reflux after unilateral ureteral reimplantation--preexistent rather than new-onset reflux. Liu, C., Chin, T., Wei, C. J. Pediatr. Surg. (1999) [Pubmed]
  20. An evaluation of the inactive mouse X chromosome in somatic cell hybrids. Salido, E.C., Passage, M.B., Yen, P.H., Shapiro, L.J., Mohandas, T.K. Somat. Cell Mol. Genet. (1993) [Pubmed]
  21. Expression of RPS4X in fibroblasts from patients with structural aberrations of the X chromosome. Just, W., Geerkens, C., Held, K.R., Vogel, W. Hum. Genet. (1992) [Pubmed]
  22. Isolation of an abundantly expressed sequence from the human X chromosome by differential screening. Wiles, M.V., Alexander, C.M., Goodfellow, P.N. Somat. Cell Mol. Genet. (1988) [Pubmed]
  23. Identification of a 40S ribosomal protein S4-derived H-Y epitope able to elicit a lymphoblast-specific cytotoxic T lymphocyte response. Ivanov, R., Aarts, T., Hol, S., Doornenbal, A., Hagenbeek, A., Petersen, E., Ebeling, S. Clin. Cancer Res. (2005) [Pubmed]
  24. Scar assessment: current problems and future solutions. Powers, P.S., Sarkar, S., Goldgof, D.B., Cruse, C.W., Tsap, L.V. The Journal of burn care & rehabilitation. (1999) [Pubmed]
  25. The effect of silicone gel sheets on perfusion of hypertrophic burn scars. Musgrave, M.A., Umraw, N., Fish, J.S., Gomez, M., Cartotto, R.C. The Journal of burn care & rehabilitation. (2002) [Pubmed]
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