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PAX genes in childhood oncogenesis: developmental biology gone awry?

Oncogene volume 34, pages 2681–2689 (2015)Cite this article

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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.

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Acknowledgements

This work was supported by funding to RLG by the Burroughs Wellcome Fund (Career Award for Medical Scientists), Alex's Lemonade Stand Foundation ("A" Award), American Cancer Society (Research Scholars Grant) and UTSW Department of Pathology (Research Grant). We thank Stephen X. Skapek for helpful suggestions regarding this manuscript.

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  1. Department of Pediatrics, University of Texas Southwestern Medical Center at Dallas, Dallas, TX, USA

    P Mahajan, P J Leavey & R L Galindo

  2. Department of Pathology, University of Texas Southwestern Medical Center at Dallas, Dallas, TX, USA

    R L Galindo

  3. Department of Molecular Biology, University of Texas Southwestern Medical Center at Dallas, Dallas, TX, USA

    R L Galindo

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  1. P Mahajan
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Correspondence to R L Galindo.

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Mahajan, P., Leavey, P. & Galindo, R. PAX genes in childhood oncogenesis: developmental biology gone awry?. Oncogene 34, 2681–2689 (2015). https://doi.org/10.1038/onc.2014.209

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