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Disease relevance of Ectromelia

  • Spina bifida and truncation of the tail and lumbosacral region (including abnormalities of the kidneys, urogenital tract, and hindgut) are the most conspicuous defects, leading in extreme cases to a sirenomelia ("mermaid tail") phenotype [1].
  • Inhibition of type 1 cytokine-mediated inflammation by a soluble CD30 homologue encoded by ectromelia (mousepox) virus [2].
  • Through the use of perforin-deficient mice, we were able to demonstrate a requirement for this molecule in the clearance of some viruses, such as ectromelia virus, whilst for others, such as vaccinia virus, perforin was less important but IFN-gamma was essential [3].
  • Adoptive transfer of ectromelia virus meningitis was most efficient when donor-immune spleen cells and recipients were compatible in the K region of the H-2 gene complex [4].
  • Here we show that vaccinia, ectromelia and cowpox viruses secrete from infected cells a soluble IL-18BP (vIL-18BP) that may modulate the host antiviral response [5].

High impact information on Ectromelia

  • Retinoic acid has profound effects on vertebrate limb morphogenesis (refs 1-6, reviewed in refs 7-9), including in the mouse, where it can act as a teratogen generating phocomelia and bone defects [6].
  • The finding of a CD30 homologue encoded by ectromelia virus suggests a role for CD30 in antiviral defense [2].
  • We infected TNF receptor (TNFR)-deficient mice with the virulent murine pathogen, ectromelia virus (EV), and observed that otherwise resistant mice were susceptible to lethal infection [7].
  • It was found that BALB/c-H-2db mice, which lack detectable cell-surface H-2L gene products, were able to generate influenza- and vaccinia-immune cytotoxic T cells which lyse D region-compatible target cells, although they have been reported to be incapable of making a similar response to ectromelia virus (7) [8].
  • Phocomelia in infant whose mother took large doses of pyridoxine during pregnancy [9].

Chemical compound and disease context of Ectromelia

  • Chromosomal studies on children with phocomelia, exposed to debendox during early pregnancy [10].
  • The observation that the protein synthesized by ectromelia virus-infected cells reacted with only one of the three MAbs provided a means of mapping the gene encoding the glycoprotein [11].
  • It was observed that expression of IL-4 by a thymidine kinase-positive ectromelia virus suppressed cytolytic responses of NK and CTL and the expression of gamma interferon by the latter [12].
  • The patterns of anomalies of the two sisters do not fit into any of the syndromes featuring phocomelia; there was no prenatal exposure to thalidomide or any other possible teratogen [13].
  • Exposure to 400 mg/kg hydroxyurea did not affect the progeny, whereas exposure to 500 or 600 mg/kg resulted in dose-dependent increases in fetal resorptions and malformations, including curly tails, abnormal limbs (oligodactyly, hemimelia, and amelia), and short ribs [14].

Biological context of Ectromelia

  • Although a number of Grzs, including GrzM, have been shown to mediate target cell apoptosis in the presence of perforin, the biological activity of Grz has been restricted to control of a number of viral pathogens, including two natural mouse pathogens, ectromelia, and murine CMV (MCMV) [15].
  • Haplotypes for a marker locus, D1Mit57, from the differential segment were determined in (D2.R4 x D2)F1 x D2 backcross mice, which were then infected with ectromelia virus [16].
  • Using point mutagenesis, we analyzed the ectromelia virus IL-18BP to identify residues involved in binding [17].
  • Maternal administration of a single dose of retinoic acid (vitamin A acid, 100 mg/kg) on either the 11th, 11 1/2, 12th, 12 1/2, 13th or 13 1/2 day of gestation produced phocomelia or partial phocomelia in ICR/DUB fetuses [18].
  • We have reported two cases of sirenomelia sequence associated with a history of cocaine exposure during a major part or the entire extent of the first trimester of pregnancy [19].

Anatomical context of Ectromelia


Gene context of Ectromelia

  • Inactivating mutations in ESCO2 cause SC phocomelia and Roberts syndrome: no phenotype-genotype correlation [24].
  • The ectromelia virus protein was found to block NF-kappaB activation and induction of IFN-gamma in response to IL-18 [5].
  • The multidrug resistance gene mdr1a influences resistance to ectromelia virus infection by mechanisms other than conventional immunity [25].
  • We employed mdr1a gene knock out (mdr1a-/-) mice and ectromelia virus (EV) to elucidate the role of P-gp in resistance to EV [25].
  • Using mice deficient for granzyme A, we show here that granzyme A plays a crucial role in recovery from the natural mouse pathogen, ectromelia, by mechanisms other than cytolytic activity [26].

Analytical, diagnostic and therapeutic context of Ectromelia

  • To determine whether this LP-BM5 MuLV infection would affect the innate resistance of B6 mice to a naturally occurring, highly virulent murine pathogen, mice were exposed to ectromelia virus at various times after treatment with LP-BM5 viruses [27].
  • The lesions imaged were fibular hemimelia (n = 5), tibial hemimelia (n = 5), and congenital constriction bands (n = 3) [28].


  1. The retinoic acid-metabolizing enzyme, CYP26A1, is essential for normal hindbrain patterning, vertebral identity, and development of posterior structures. Abu-Abed, S., Dollé, P., Metzger, D., Beckett, B., Chambon, P., Petkovich, M. Genes Dev. (2001) [Pubmed]
  2. Inhibition of type 1 cytokine-mediated inflammation by a soluble CD30 homologue encoded by ectromelia (mousepox) virus. Saraiva, M., Smith, P., Fallon, P.G., Alcami, A. J. Exp. Med. (2002) [Pubmed]
  3. Cytokines and immunity to viral infections. Ramshaw, I.A., Ramsay, A.J., Karupiah, G., Rolph, M.S., Mahalingam, S., Ruby, J.C. Immunol. Rev. (1997) [Pubmed]
  4. The role of the major histocompatibility complex in the adoptive transfer of ectromelia virus meningitis. Berger, M.L. J. Neuropathol. Exp. Neurol. (1982) [Pubmed]
  5. Ectromelia, vaccinia and cowpox viruses encode secreted interleukin-18-binding proteins. Smith, V.P., Bryant, N.A., Alcamí, A. J. Gen. Virol. (2000) [Pubmed]
  6. Differential expression of genes encoding alpha, beta and gamma retinoic acid receptors and CRABP in the developing limbs of the mouse. Dollé, P., Ruberte, E., Kastner, P., Petkovich, M., Stoner, C.M., Gudas, L.J., Chambon, P. Nature (1989) [Pubmed]
  7. Antiviral activity of tumor necrosis factor (TNF) is mediated via p55 and p75 TNF receptors. Ruby, J., Bluethmann, H., Peschon, J.J. J. Exp. Med. (1997) [Pubmed]
  8. Involvement of H-2L gene products in virus-immune T-cell recognition. Evidence for an H-2L-restricted T-cell response. Biddison, W.E., Hansen, T.H., Levy, R.B., Doherty, P.C. J. Exp. Med. (1978) [Pubmed]
  9. Phocomelia in infant whose mother took large doses of pyridoxine during pregnancy. Gardner, L.I., Welsh-Sloan, J., Cady, R.B. Lancet (1985) [Pubmed]
  10. Chromosomal studies on children with phocomelia, exposed to debendox during early pregnancy. Hughes, D.T., Cavanagh, N. Lancet (1983) [Pubmed]
  11. Extracellular vaccinia virus envelope glycoprotein encoded by the A33R gene. Roper, R.L., Payne, L.G., Moss, B. J. Virol. (1996) [Pubmed]
  12. Expression of mouse interleukin-4 by a recombinant ectromelia virus suppresses cytolytic lymphocyte responses and overcomes genetic resistance to mousepox. Jackson, R.J., Ramsay, A.J., Christensen, C.D., Beaton, S., Hall, D.F., Ramshaw, I.A. J. Virol. (2001) [Pubmed]
  13. Phocomelia and additional anomalies in two sisters. Schinzel, A. Hum. Genet. (1990) [Pubmed]
  14. Activator protein-1 (AP-1) DNA binding activity is induced by hydroxyurea in organogenesis stage mouse embryos. Yan, J., Hales, B.F. Toxicol. Sci. (2005) [Pubmed]
  15. Functional analysis of granzyme M and its role in immunity to infection. Pao, L.I., Sumaria, N., Kelly, J.M., van Dommelen, S., Cretney, E., Wallace, M.E., Anthony, D.A., Uldrich, A.P., Godfrey, D.I., Papadimitriou, J.M., Mullbacher, A., Degli-Esposti, M.A., Smyth, M.J. J. Immunol. (2005) [Pubmed]
  16. Chromosome mapping of Rmp-4, a gonad-dependent gene encoding host resistance to mousepox. Brownstein, D.G., Gras, L. J. Virol. (1995) [Pubmed]
  17. Identification of residues in an orthopoxvirus interleukin-18 binding protein involved in ligand binding and species specificity. Esteban, D.J., Buller, R.M. Virology (2004) [Pubmed]
  18. Production of congenital limb defects with retinoic acid: phenomenological evidence of progressive differentiation during limb morphogenesis. Kwasigroch, T.E., Kochhar, D.M. Anat. Embryol. (1980) [Pubmed]
  19. Sirenomelia accompanying exposure of the embryo to cocaine. Sarpong, S., Headings, V. South. Med. J. (1992) [Pubmed]
  20. A poxvirus protein that binds to and inactivates IL-18, and inhibits NK cell response. Born, T.L., Morrison, L.A., Esteban, D.J., VandenBos, T., Thebeau, L.G., Chen, N., Spriggs, M.K., Sims, J.E., Buller, R.M. J. Immunol. (2000) [Pubmed]
  21. Pathogenesis of caudal dysgenesis/sirenomelia induced by ochratoxin A in chick embryos. Wei, X., Sulik, K.K. Teratology (1996) [Pubmed]
  22. Influence of cyclophosphamide and 4-ketocyclophosphamide on mouse limb development. Manson, J.M., Smith, C.C. Teratology (1977) [Pubmed]
  23. Pattern of skeletal malformations produced by Dominant hemimelia (Dh). Morin, B.J., Owen, M.H., Ramamurthy, G.V., Holmes, L.B. Teratology (1999) [Pubmed]
  24. Inactivating mutations in ESCO2 cause SC phocomelia and Roberts syndrome: no phenotype-genotype correlation. Schüle, B., Oviedo, A., Johnston, K., Pai, S., Francke, U. Am. J. Hum. Genet. (2005) [Pubmed]
  25. The multidrug resistance gene mdr1a influences resistance to ectromelia virus infection by mechanisms other than conventional immunity. Xu, D., Regner, M., Smith, D., Ruby, J., Johnstone, R., Müllbacher, A. Immunol. Cell Biol. (2004) [Pubmed]
  26. Granzyme A is critical for recovery of mice from infection with the natural cytopathic viral pathogen, ectromelia. Müllbacher, A., Ebnet, K., Blanden, R.V., Hla, R.T., Stehle, T., Museteanu, C., Simon, M.M. Proc. Natl. Acad. Sci. U.S.A. (1996) [Pubmed]
  27. Abrogation of resistance to severe mousepox in C57BL/6 mice infected with LP-BM5 murine leukemia viruses. Buller, R.M., Yetter, R.A., Fredrickson, T.N., Morse, H.C. J. Virol. (1987) [Pubmed]
  28. MR imaging in congenital lower limb deformities. Laor, T., Jaramillo, D., Hoffer, F.A., Kasser, J.R. Pediatric radiology. (1996) [Pubmed]
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