PAX genes in childhood oncogenesis: Developmental biology gone awry?

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Abstract

Childhood solid tumors often arise from embryonal-like cells, which are distinct from the epithelial cancers observed in adults, and etiologically can be considered as 'developmental patterning gone awry'. Paired-box (PAX) genes encode a family of evolutionarily conserved transcription factors that are important regulators of cell lineage specification, migration and tissue patterning. PAX loss-of-function mutations are well known to cause potent developmental phenotypes in animal models and underlie genetic disease in humans, whereas dysregulation and/or genetic modification of PAX genes have been shown to function as critical triggers for human tumorigenesis. Consequently, exploring PAX-related pathobiology generates insights into both normal developmental biology and key molecular mechanisms that underlie pediatric cancer, which are the topics of this review.

Original languageEnglish (US)
Pages (from-to)2681-2689
Number of pages9
JournalOncogene
Volume34
Issue number21
DOIs
Publication statusPublished - May 21 2015

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ASJC Scopus subject areas

  • Molecular Biology
  • Cancer Research
  • Genetics

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