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. 1993 Apr 15;90(8):3192–3196. doi: 10.1073/pnas.90.8.3192

Transgenic mouse model for neurocristopathy: Schwannomas and facial bone tumors.

N A Jensen 1, M L Rodriguez 1, J S Garvey 1, C A Miller 1, L Hood 1
PMCID: PMC46265  PMID: 8386366

Abstract

We have characterized a strain of double transgenic mice with simian virus 40 large tumor antigen and prokaryotic lacZ under the control of the myelin basic protein promoter that develops spindle-cell sarcomas and osteogenic sarcomas at 5-7 months of age. Although poorly differentiated, the spindle-cell sarcomas were characterized as malignant Schwannomas based on their neural association, the presence of basal lamina, and expression of Schwann cell-specific genes. The osteogenic sarcomas were often multiple and appeared predominantly in the facial bones, less frequently in the ribs and vertebral column, and only rarely in the appendicular skeleton. Benign osteoblastic lesions were often observed adjacent to these sarcomas. Both the osteoblastic cells in the facial skeleton and Schwann cells are regarded as neural crest derivatives. The biological properties and anatomical location of these tumors suggest that they may share a common origin from the neural crest or its derivatives. R.P. Bolande [Hum. Pathol. (1974) 5, 409-429] introduced the term neurocristopathy as a unifying concept to describe such lesions arising from the neural crest or its derivatives. Cell lines established from both bone and Schwann cell tumors arising in these transgenic mice express simian virus 40 large tumor antigen mRNA as well as functional large tumor antigen. Such cell lines are potentially valuable in the search for markers that identify mammalian neural crest derivatives.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Adams J. M., Cory S. Transgenic models of tumor development. Science. 1991 Nov 22;254(5035):1161–1167. doi: 10.1126/science.1957168. [DOI] [PubMed] [Google Scholar]
  2. Baroffio A., Dupin E., Le Douarin N. M. Common precursors for neural and mesectodermal derivatives in the cephalic neural crest. Development. 1991 May;112(1):301–305. doi: 10.1242/dev.112.1.301. [DOI] [PubMed] [Google Scholar]
  3. Gandelman K. Y., Pfeiffer S. E., Carson J. H. Cyclic AMP regulation of P0 glycoprotein and myelin basic protein gene expression in semi-differentiated peripheral neurinoma cell line D6P2T. Development. 1989 Jun;106(2):389–398. doi: 10.1242/dev.106.2.389. [DOI] [PubMed] [Google Scholar]
  4. Harlow E., Crawford L. V., Pim D. C., Williamson N. M. Monoclonal antibodies specific for simian virus 40 tumor antigens. J Virol. 1981 Sep;39(3):861–869. doi: 10.1128/jvi.39.3.861-869.1981. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Harlow E., Pim D. C., Crawford L. V. Complex of simian virus 40 large-T antigen and host 53,000-molecular-weight protein in monkey cells. J Virol. 1981 Feb;37(2):564–573. doi: 10.1128/jvi.37.2.564-573.1981. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Jensen N. A., Smith G. M., Shine H. D., Garvey J. S., Hood L. Distinct hypomyelinated phenotypes in MBP-SV40 large T transgenic mice. J Neurosci Res. 1993 Feb 15;34(3):257–264. doi: 10.1002/jnr.490340302. [DOI] [PubMed] [Google Scholar]
  7. Keynes R. J., Stern C. D. Mechanisms of vertebrate segmentation. Development. 1988 Jul;103(3):413–429. doi: 10.1242/dev.103.3.413. [DOI] [PubMed] [Google Scholar]
  8. Lane D. P. Cancer. p53, guardian of the genome. Nature. 1992 Jul 2;358(6381):15–16. doi: 10.1038/358015a0. [DOI] [PubMed] [Google Scholar]
  9. Le Douarin N. M., Smith J. Development of the peripheral nervous system from the neural crest. Annu Rev Cell Biol. 1988;4:375–404. doi: 10.1146/annurev.cb.04.110188.002111. [DOI] [PubMed] [Google Scholar]
  10. Le Douarin N., Dulac C., Dupin E., Cameron-Curry P. Glial cell lineages in the neural crest. Glia. 1991;4(2):175–184. doi: 10.1002/glia.440040209. [DOI] [PubMed] [Google Scholar]
  11. Lemke G. Unwrapping the genes of myelin. Neuron. 1988 Sep;1(7):535–543. doi: 10.1016/0896-6273(88)90103-1. [DOI] [PubMed] [Google Scholar]
  12. Monuki E. S., Kuhn R., Weinmaster G., Trapp B. D., Lemke G. Expression and activity of the POU transcription factor SCIP. Science. 1990 Sep 14;249(4974):1300–1303. doi: 10.1126/science.1975954. [DOI] [PubMed] [Google Scholar]
  13. Monuki E. S., Weinmaster G., Kuhn R., Lemke G. SCIP: a glial POU domain gene regulated by cyclic AMP. Neuron. 1989 Dec;3(6):783–793. doi: 10.1016/0896-6273(89)90247-x. [DOI] [PubMed] [Google Scholar]
  14. Sobue G., Shuman S., Pleasure D. Schwann cell responses to cyclic AMP: proliferation, change in shape, and appearance of surface galactocerebroside. Brain Res. 1986 Jan 1;362(1):23–32. doi: 10.1016/0006-8993(86)91394-6. [DOI] [PubMed] [Google Scholar]
  15. Verbout A. J. A critical review of the 'neugliederung' concept in relation to the development of the vertebral column. Acta Biotheor. 1976;25(4):219–258. doi: 10.1007/BF00046818. [DOI] [PubMed] [Google Scholar]

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