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Mouse models of childhood cancer of the nervous system
  1. M A Dyer
  1. Correspondence to:
 Dr M A Dyer
 St Jude Children’s Research Hospital, Department of Developmental Neurobiology, Memphis, TN 38105, USA; michael.dyerstjude.org; http://www.stjude.org/dyer/

Abstract

Targeted cancer treatments rely on understanding signalling cascades, genetic changes, and compensatory programmes activated during tumorigenesis. Increasingly, pathologists are required to interpret molecular profiles of tumour specimens to target new treatments. This is challenging because cancer is a heterogeneous disease—tumours change over time in individual patients and genetic lesions leading from preneoplasia to malignancy can differ substantially between patients. For childhood tumours of the nervous system, the challenge is even greater, because tumours arise from progenitor cells in a developmental context different from that of the adult, and the cells of origin, neural progenitor cells, show considerable temporal and spatial heterogeneity during development. Thus, the underlying mechanisms regulating normal development of the nervous system also need to be understood. Many important advances have come from model mouse genetic systems. This review will describe several mouse models of childhood tumours of the nervous system, emphasising how understanding the normal developmental processes, combined with mouse models of cancer and the molecular pathology of the human diseases, can provide the information needed to treat cancer more effectively.

  • retinoblastoma
  • medulloblastoma
  • neuroblastoma
  • retrovirus
  • xenograft
  • APC, adenomatous polyposis coli
  • CNS, central nervous system
  • E, embryonic day
  • EGL, external germinal layer
  • GSK-3β, glycogen synthase kinase 3β
  • Lig4, DNA ligase IV
  • P, postnatal day
  • PARP, poly (ADP-ribose) polymerase
  • SCID, severe combined immunodeficiency disorder
  • TCF/LEF, T cell factor/lymphoid enhancer factor

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