title: Cell lines from MYCN transgenic murine tumours reflect the molecular and biological characteristics of human neuroblastoma.
abstract: Overexpression of the human MYCN oncogene driven by a tyrosine hydroxylase promoter causes tumours in transgenic mice that recapitulate the childhood cancer neuroblastoma. To establish an in vitro model to study this process, a series of isogenic cell lines were developed from these MYCN-driven murine tumours. Lines were established from tumours arising in homozygous and hemizygous MYCN transgenic mice. Hemizygous tumours gave rise to cell lines growing only in suspension. Homozygous tumours gave rise to similar suspension lines as well as morphologically distinct substrate-adherent lines characteristic of human S-type neuroblastoma cells. FISH analysis demonstrated selective MYCN transgene amplification in cell lines derived from hemizygous mice. Comparative genomic hybridisation (CGH) and fluorescence in situ hybridisation (FISH) analysis confirmed a range of neuroblastoma-associated genetic changes in the various lines, in particular, gain of regions syntenic with human 17q. These isogenic lines together with the transgenic mice thus represent valuable models for investigating the biological characteristics of aggressive neuroblastoma.
authors: Children's Cancer Institute Australia for Medical Research, P.O. Box 81, Randwick, 2031 Sydney, Australia.
authors: Andy J
authors: AJ
authors: Cheng
authors: Ngan Ching
authors: NC
authors: Cheng
authors: Jette
authors: J
authors: Ford
authors: Janice
authors: J
authors: Smith
authors: Jayne E
authors: JE
authors: Murray
authors: Claudia
authors: C
authors: Flemming
authors: Maria
authors: M
authors: Lastowska
authors: Michael S
authors: MS
authors: Jackson
authors: Christopher S
authors: CS
authors: Hackett
authors: William A
authors: WA
authors: Weiss
authors: Glenn M
authors: GM
authors: Marshall
authors: Ursula R
authors: UR
authors: Kees
authors: Murray D
authors: MD
authors: Norris
authors: Michelle
authors: M
authors: Haber
mesh_terms: D000818:Animals; D045744:Cell Line, Tumor; D002869:Chromosome Aberrations; D015870:Gene Expression; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D007621:Karyotyping; D051379:Mice; D008807:Mice, Inbred BALB C; D008819:Mice, Nude; D008822:Mice, Transgenic; D000071447:N-Myc Proto-Oncogene Protein; D009368:Neoplasm Transplantation; D009447:Neuroblastoma / Q000235:genetics*; D009687:Nuclear Proteins / Q000235:genetics*; D015513:Oncogene Proteins / Q000235:genetics*; D011003:Ploidies
chemical_list: C459801:MYCN protein, human; D000071447:N-Myc Proto-Oncogene Protein; D009687:Nuclear Proteins; D015513:Oncogene Proteins