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Notch Signaling in Pulmonary Hypertension

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Membrane Receptors, Channels and Transporters in Pulmonary Circulation

Part of the book series: Advances in Experimental Medicine and Biology ((volume 661))

Abstract

Proteins of the Notch receptor family are cell surface receptors that transduce signals between neighboring cells. The Notch signaling pathway is highly evolutionarily conserved and critical for cell fate determination during embryogenesis and early postnatal life, including many aspects of vascular development. The interaction of Notch receptor with its membrane-bound ligands leads to cleavage of the receptor into an intracellular domain that translocates to the nucleus and activates the transcription factor, C-promoter binding factor 1 (CBF1; also known as Recombination signal-binding protein for immunoglobulin κ J region, RBPJ). To date, four Notch receptors have been characterized in humans. Of these, Notch3 is expressed only in arterial smooth muscle cells in the human. The functional importance of Notch3 signaling in human vascular smooth muscle cells has been recognized. Notch3 receptor signaling has been shown in several model systems to control vascular smooth muscle cell proliferation and maintain smooth muscle cells in an undifferentiated state. This review focuses on recent findings of the role of Notch3 in regulating vascular smooth muscle cell behavior and phenotype and discusses the potential role of Notch3 signaling in the genesis of pulmonary arterial hypertension.

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Authors and Affiliations

  1. Division of Cardiothoracic Surgery, University of California, San Diego, CA, 92103-8892, USA

    Patricia A. Thistlethwaite, Xiaodong Li & Xiaoxue Zhang

Authors
  1. Patricia A. Thistlethwaite
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  2. Xiaodong Li
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  3. Xiaoxue Zhang
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Corresponding author

Correspondence to Patricia A. Thistlethwaite .

Editor information

Editors and Affiliations

  1. Departments of Medicine and Pharmacology, University of Illinois at Chicago, Chicago, 60612, USA

    Jason X. -J. Yuan

  2. Guy’s Hospital Campus, Dept. Physiology, King’s College London, London, SE1 1UL, United Kingdom

    Jeremy P. T. Ward

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© 2010 Humana Press, a part of Springer Science+Business Media, LLC

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Thistlethwaite, P.A., Li, X., Zhang, X. (2010). Notch Signaling in Pulmonary Hypertension. In: Yuan, JJ., Ward, J. (eds) Membrane Receptors, Channels and Transporters in Pulmonary Circulation. Advances in Experimental Medicine and Biology, vol 661. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60761-500-2_18

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