title: Transdifferentiation of endothelial cells to smooth muscle cells play an important role in vascular remodelling.
abstract: Pulmonary artery remodelling it is a major feature of pulmonary hypertension (PH). It is characterised by cellular and structural changes of the pulmonary arteries causing higher pulmonar vascular resistance and right ventricular failure. Abnormal deposition of smooth muscle-like (SM-like) cells in normally non-muscular, small diameter vessels and a deregulated control of endothelial cells are considered pathological features of PH. The origin of the SM-like cells and the mechanisms underlying the development and progression of this remodelling process are not understood. Endothelial cells within the intima may migrate from their organised layer of cells and transition to mesenchymal or SM-like phenotype in a process called endothelial-mesenchymal transition (EnMT). Traditionally, Waddington's epigenetic landscape illustrates that fates of somatic cells are progressively determined to compulsorily follow a downhill differentiation pathway. EnMT induces the transformation of cells with stem cell traits, therefore contrasting Waddington's theory and confirming that cell fate seems to be far more flexible than previously thought. The prospect of therapeutic inhibition of EnMT to delay or prevent PH may represent a promising new treatment modality.
authors.affiliation: Department of Pulmonary Medicine, Hospital Clínic-Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona Barcelona 08036, Spain.
authors.forename: Núria
authors.initials: N
authors.lastname: Coll-Bonfill
authors.affiliation: Department of Pulmonary Medicine, Hospital Clínic-Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona Barcelona 08036, Spain ; Instituto de Investigación Médica M, Y M. Ferreyra, INIMEC-CONICET, Universidad Nacional de Córdoba Argentina.
authors.forename: Melina Mara
authors.initials: MM
authors.lastname: Musri
authors.affiliation: Department of Pulmonary Medicine, Hospital Clínic-Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona Barcelona 08036, Spain ; Centro de Investigación Biomédica en Red de Enfermedades Respiratorias Spain.
authors.forename: Victor
authors.initials: V
authors.lastname: Ivo
authors.affiliation: Department of Pulmonary Medicine, Hospital Clínic-Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona Barcelona 08036, Spain ; Centro de Investigación Biomédica en Red de Enfermedades Respiratorias Spain.
authors.forename: Joan Albert
authors.initials: JA
authors.lastname: Barberà
authors.affiliation: Department of Pulmonary Medicine, Hospital Clínic-Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona Barcelona 08036, Spain ; Centro de Investigación Biomédica en Red de Enfermedades Respiratorias Spain.
authors.forename: Olga
authors.initials: O
authors.lastname: Tura-Ceide
keywords: Endothelial to mesenchymal transition; cellular reprogramming; endothelial cells; pulmonary hypertension; remodelling