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Current Advances in Osteosarcoma pp 129–145Cite as

RECQ DNA Helicases and Osteosarcoma

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Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 804))

Abstract

The RECQ family of DNA helicases is a conserved group of enzymes that are important for maintaining genomic integrity. In humans, there are five RECQ helicase genes, and mutations in three of them—BLM, WRN, and RECQL4—are associated with the genetic disorders Bloom syndrome, Werner syndrome, and Rothmund–Thomson syndrome (RTS), respectively. Importantly all three diseases are cancer predisposition syndromes. Patients with RTS are highly and uniquely susceptible to developing osteosarcoma; thus, RTS provides a good model to study the pathogenesis of osteosarcoma. The “tumor suppressor” role of RECQL4 and the other RECQ helicases is an area of active investigation. This chapter reviews what is currently known about the cellular functions of RECQL4 and how these may relate to tumorigenesis, as well as ongoing efforts to understand RECQL4’s functions in vivo using animal models. Understanding the RECQ pathways may provide insight into avenues for novel cancer therapies in the future.

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Authors and Affiliations

  1. Section of Hematology/Oncology, Department of Pediatrics, Texas Children’s Cancer Center, Baylor College of Medicine, 1102 Bates Avenue, Suite 1200, Houston, TX, 77030, USA

    Linchao Lu Ph.D., Weidong Jin M.S. & Lisa L. Wang M.D.

  2. Section of Hematology/Oncology, Department of Medicine, Division of Biostatistics, Dan L.Duncan Cancer Center, Baylor College of Medicine, One Baylor Plaza, Houston, TX, USA

    Hao Liu Ph.D.

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  1. Linchao Lu Ph.D.
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  2. Weidong Jin M.S.
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  3. Hao Liu Ph.D.
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  4. Lisa L. Wang M.D.
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Correspondence to Lisa L. Wang M.D. .

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  1. Division of Pediatrics, Department of Cancer Biology, The Mary V. and John A. Reilly Distinguished Chair, University of Texas M.D. Anderson Cancer Center, Houston, Texas, USA

    Eugenie S. Kleinerman, M.D.

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Lu, L., Jin, W., Liu, H., Wang, L.L. (2014). RECQ DNA Helicases and Osteosarcoma. In: Kleinerman, M.D., E. (eds) Current Advances in Osteosarcoma. Advances in Experimental Medicine and Biology, vol 804. Springer, Cham. https://doi.org/10.1007/978-3-319-04843-7_7

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