A Notch3-Marked Subpopulation of Vascular Smooth Muscle Cells Is the Cell of Origin for Occlusive Pulmonary Vascular Lesions | Zendy

L.C. Steffes | Zendy; Alexis A. Froistad | Zendy; Adam Andruska | Zendy; Mario Boehm | Zendy; Madeleine McGlynn | Zendy; Fan Zhang | Zendy; Wenming Zhang | Zendy; David Hou | Zendy; Xuefei Tian | Zendy; Lucile Miquerol | Zendy; Kari C. Nadeau | Zendy; Ross J. Metzger | Zendy; Edda Spiekerkoetter | Zendy; Maya E. Kumar | Zendy

Open Access

A Notch3-Marked Subpopulation of Vascular Smooth Muscle Cells Is the Cell of Origin for Occlusive Pulmonary Vascular Lesions

Author(s) -

L.C. Steffes,

Alexis A. Froistad,

Adam Andruska,

Mario Boehm,

Madeleine McGlynn,

Fan Zhang,

Wenming Zhang,

David Hou,

Xuefei Tian,

Lucile Miquerol,

Kari C. Nadeau,

Ross J. Metzger,

Edda Spiekerkoetter,

Maya E. Kumar

Publication year - 2020

Publication title -

circulation

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 7.795

H-Index - 607

eISSN - 1524-4539

pISSN - 0009-7322

DOI - 10.1161/circulationaha.120.045750

Subject(s) - medicine , vascular smooth muscle , occlusive , pulmonary vasculature , vascular disease , cardiology , anatomy , pathology , smooth muscle , pulmonary artery

Pulmonary arterial hypertension (PAH) is a fatal disease characterized by profound vascular remodeling in which pulmonary arteries narrow because of medial thickening and occlusion by neointimal lesions, resulting in elevated pulmonary vascular resistance and right heart failure. Therapies targeting the neointima would represent a significant advance in PAH treatment; however, our understanding of the cellular events driving neointima formation, and the molecular pathways that control them, remains limited.

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