BMP Signaling and Beyond: Breaking the Cell Code of PAH

Dear Colleagues,

Pulmonary arterial hypertension (PAH) is a disease associated with abnormally elevated pulmonary pressures and right heart failure that, if untreated, leads to premature death. Despite the availability of 14 approved drugs, PAH remains a challenging disease with a high index of morbidity and mortality. At the center of this dilemma is the progressive nature of the disease, which ultimately overcomes medical therapy and leaves the patient with few options for life-saving interventions.

A key feature of PAH is the rich milieu of cell types and molecular markers found within the vascular lesions. Advances in genetics and molecular biology have revolutionized our understanding of the mechanisms behind PAH pathogenesis and the genetic modifiers that increase the risk of disease development on susceptible individuals. For over 20 years, the BMP pathway has remained a subject of intense research given its association with both hereditary and sporadic PAH and has led to the development of medications tailored to restore BMP homeostasis in the pulmonary vasculature. However, despite these exciting advances, there are still open questions regarding the cellular origin of PAH and whether dysregulation of BMP signaling alone is responsible for driving the abnormal cellular changes intrinsic to the disease.

This Research Topic represents a concerted effort to update the community on the state-of-the-art knowledge in PAH and to discuss provocative questions that remain of great interest to investigators in the field. We hope to provide readers with a unique and unbiased resource that will be useful in the development of new research directions that will guide efforts to identify new treatment targets and biomarkers for PAH.

Dr. Vinicio A. De Jesus Perez
Dr. James West
Dr. Jair Antonio Tenorio Castaño
Guest Editors

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