Endothelial cells and pulmonary arterial hypertension: apoptosis, proliferation, interaction and transdifferentiation.
Severe pulmonary arterial hypertension, whether idiopathic or secondary, is characterized by structural alterations of microscopically small pulmonary arterioles. The vascular lesions in this group of pulmonary hypertensive diseases show actively proliferating endothelial cells without evidence of apoptosis. In this article, we review pathogenetic concepts of severe pulmonary arterial hypertension and explain the term "complex vascular lesion ", commonly named "plexiform lesion", with endothelial cell dysfunction, i.e., apoptosis, proliferation, interaction with smooth muscle cells and transdifferentiation.
Sakao
Seiichiro
S
Department of Respirology (B2), Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan. sakaos@faculty.chiba-u.jp
Tatsumi
Koichiro
K
Voelkel
Norbert F
NF
D000818:Animals; D017209:Apoptosis / Q000235:genetics; D002450:Cell Communication / Q000235:genetics; D049109:Cell Proliferation; D054338:Cell Transdifferentiation / Q000235:genetics; D018450:Disease Progression; D042783:Endothelial Cells / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000235:genetics / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D009131:Muscle, Smooth, Vascular / Q000378:metabolism / Q000473:pathology; D011651:Pulmonary Artery / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D012720:Severity of Illness Index; D015398:Signal Transduction