The BMP Receptor 2 in Pulmonary Arterial Hypertension: When and Where the Animal Model Matches the Patient.

Cells
Chris HappéMarie-José Goumans

Abstract

Background: Mutations in bone morphogenetic protein receptor type II (BMPR2) are leading to the development of hereditary pulmonary arterial hypertension (PAH). In non-hereditary forms of PAH, perturbations in the transforming growth factor-β (TGF-β)/BMP-axis are believed to cause deficient BMPR2 signaling by changes in receptor expression, the activity of the receptor and/or downstream signaling. To date, BMPR2 expression and its activity in the lungs of patients with non-hereditary PAH is poorly characterized. In recent decades, different animal models have been used to understand the role of BMPR2 signaling in PAH pathophysiology. Specifically, the monocrotaline (MCT) and Sugen-Hypoxia (SuHx) models are extensively used in interventional studies to examine if restoring BMPR2 signaling results in PAH disease reversal. While PAH is assumed to develop in patients over months or years, pulmonary hypertension in experimental animal models develops in days or weeks. It is therefore likely that modifications in BMP and TGF-β signaling in these models do not fully recapitulate those in patients. In order to determine the translational potential of the MCT and SuHx models, we analyzed the BMPR2 expression and activity in the lungs of...Continue Reading

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Citations

Sep 5, 2020·Hypertension Research : Official Journal of the Japanese Society of Hypertension·Michiel Alexander de RaafHarm Jan Bogaard
Oct 31, 2020·Cells·Isabel FabregatAránzazu Sánchez
Jan 14, 2021·Biomedicines·Kondababu KurakulaMarie-José Goumans
Oct 7, 2020·American Journal of Respiratory and Critical Care Medicine·Jason HongXia Yang
Aug 26, 2021·Communications Biology·Tatyana NovoyatlevaRalph T Schermuly
Aug 28, 2021·International Journal of Molecular Sciences·José L Sánchez-GloriaHoracio Osorio-Alonso

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Methods Mentioned

BETA
protein assay
dissection

Software Mentioned

Slidebook
Adobe Illustrator
FIJI
GraphPad Prism

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