The promoter of the human cystic fibrosis transmembrane conductance regulator gene directing SV40 T antigen expression induces malignant proliferation of ependymal cells in transgenic mice

F. Perraud, K. Yoshimura, B. Louis, W. Dalemans, D. Ali-Hadji, H. Schultz, M. C. Claudepierre, C. Chartier, C. Danel, J. P. Bellocq, Ronald Crystal, J. P. Lecocq, A. Pavirani

Research output: Contribution to journalArticle

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Abstract

Transgenic mice bearing a human cystic fibrosis transmembrane conductance regulator (CFTR) promoter-SV40 T antigen fusion transgene were generated in order to localize in vivo the potential oncogenesis linked to the tissue-specific activity of the promoter for the CFTR gene. Surprisingly, the only site of tumors resulting from expression of the reporter one gene was ependymal cells lining the brain ventricles. SV40 T antigen expression in these cells led to a consistent pathology in the first weeks of age: ependymoma and consequent hydrocephaly. Tumor-derived cell lines were established, characterized and shown to originate from SV40 T antigen-induced ependymoma. No pathological alterations were found in other organs, such as lungs and pancreas, in which cystic fibrosis is pathologically manifest in humans. Such transgenic mice and derived cell lines may represent valid models for analysing (1) the role of SV40 T antigen in ependymoma formation and (2) CFTR function in ependymal cells.

Original languageEnglish
Pages (from-to)993-997
Number of pages5
JournalOncogene
Volume7
Issue number5
Publication statusPublished - 1 Jan 1992
Externally publishedYes

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Polyomavirus Transforming Antigens
Cystic Fibrosis Transmembrane Conductance Regulator
Regulator Genes
Ependymoma
Transgenic Mice
Cell Proliferation
Hydrocephalus
Tumor Cell Line
Transgenes
Reporter Genes
Cystic Fibrosis
Pancreas
Carcinogenesis
Pathology
Cell Line
Lung
Brain
Neoplasms

ASJC Scopus subject areas

  • Molecular Biology
  • Genetics
  • Cancer Research

Cite this

The promoter of the human cystic fibrosis transmembrane conductance regulator gene directing SV40 T antigen expression induces malignant proliferation of ependymal cells in transgenic mice. / Perraud, F.; Yoshimura, K.; Louis, B.; Dalemans, W.; Ali-Hadji, D.; Schultz, H.; Claudepierre, M. C.; Chartier, C.; Danel, C.; Bellocq, J. P.; Crystal, Ronald; Lecocq, J. P.; Pavirani, A.

In: Oncogene, Vol. 7, No. 5, 01.01.1992, p. 993-997.

Research output: Contribution to journalArticle

Perraud, F, Yoshimura, K, Louis, B, Dalemans, W, Ali-Hadji, D, Schultz, H, Claudepierre, MC, Chartier, C, Danel, C, Bellocq, JP, Crystal, R, Lecocq, JP & Pavirani, A 1992, 'The promoter of the human cystic fibrosis transmembrane conductance regulator gene directing SV40 T antigen expression induces malignant proliferation of ependymal cells in transgenic mice', Oncogene, vol. 7, no. 5, pp. 993-997.
Perraud, F. ; Yoshimura, K. ; Louis, B. ; Dalemans, W. ; Ali-Hadji, D. ; Schultz, H. ; Claudepierre, M. C. ; Chartier, C. ; Danel, C. ; Bellocq, J. P. ; Crystal, Ronald ; Lecocq, J. P. ; Pavirani, A. / The promoter of the human cystic fibrosis transmembrane conductance regulator gene directing SV40 T antigen expression induces malignant proliferation of ependymal cells in transgenic mice. In: Oncogene. 1992 ; Vol. 7, No. 5. pp. 993-997.
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