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Bone Morphogenetic Protein Signaling in Pulmonary Arterial Hypertension

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Bone Morphogenetic Proteins: Systems Biology Regulators

Part of the book series: Progress in Inflammation Research ((PIR))

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Abstract

A wealth of evidence from human genetics, developmental and cell biology, and translational science has implicated members of the bone morphogenetic protein (BMP) and transforming growth factor-β (TGF-β ) signaling family in the pathogenesis of pulmonary arterial hypertension (PAH). The discovery of loss-of-function germline mutations in BMPR2 and in functionally related BMP/TGF-β signal transduction molecules as causes of heritable PAH and several overlapping congenital vascular syndromes has catalyzed work to elucidate how BMP signals critically regulate vascular development, vascular homeostasis, inflammation, metabolism and pathogenic remodeling. This work in vascular biology and experimental medicine has in turn led to a more nuanced understanding by which the structurally diverse family of BMP ligands and receptors achieve their tissue-specific and context-dependent functions. Recently this work has shed light on promising new strategies by which dysregulated BMP/TGF-β might be modulated for therapeutic benefit in PAH and related conditions.

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Authors and Affiliations

  1. Brigham and Women’s Hospital, Division of Cardiovascular Medicine, Department of Medicine, 20 Shattuck Street, Thorn Biosciences 1203, Boston, MA, 02115, USA

    Peiran Yang PhD & Paul B. Yu MD, PhD, FAHA

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  1. Peiran Yang PhD
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  2. Paul B. Yu MD, PhD, FAHA
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Correspondence to Paul B. Yu MD, PhD, FAHA .

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  1. School of Medicine, University of Zagreb, Zagreb, Croatia

    Slobodan Vukicevic

  2. perForm Biologics Inc, Holliston, Massachusetts, USA

    Kuber T. Sampath

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Yang, P., Yu, P.B. (2017). Bone Morphogenetic Protein Signaling in Pulmonary Arterial Hypertension. In: Vukicevic, S., Sampath, K. (eds) Bone Morphogenetic Proteins: Systems Biology Regulators. Progress in Inflammation Research. Springer, Cham. https://doi.org/10.1007/978-3-319-47507-3_13

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