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- Title
Inactivating the Uninhibited: The Tale of Activins and Inhibins in Pulmonary Arterial Hypertension.
- Authors
Ryanto, Gusty Rizky Teguh; Musthafa, Ahmad; Hara, Tetsuya; Emoto, Noriaki
- Abstract
Advances in technology and biomedical knowledge have led to the effective diagnosis and treatment of an increasing number of rare diseases. Pulmonary arterial hypertension (PAH) is a rare disorder of the pulmonary vasculature that is associated with high mortality and morbidity rates. Although significant progress has been made in understanding PAH and its diagnosis and treatment, numerous unanswered questions remain regarding pulmonary vascular remodeling, a major factor contributing to the increase in pulmonary arterial pressure. Here, we discuss the role of activins and inhibins, both of which belong to the TGF-β superfamily, in PAH development. We examine how these relate to signaling pathways implicated in PAH pathogenesis. Furthermore, we discuss how activin/inhibin-targeting drugs, particularly sotatercep, affect pathophysiology, as these target the afore-mentioned specific pathway. We highlight activin/inhibin signaling as a critical mediator of PAH development that is to be targeted for therapeutic gain, potentially improving patient outcomes in the future.
- Subjects
PULMONARY arterial hypertension; VASCULAR remodeling; ACTIVIN; PULMONARY circulation; RARE diseases; CELLULAR signal transduction
- Publication
International Journal of Molecular Sciences, 2023, Vol 24, Issue 4, p3332
- ISSN
1661-6596
- Publication type
Article
- DOI
10.3390/ijms24043332