[Cellular signalling pathways and pathophysiology of pulmonary arterial hypertension].
BACKGROUND
Pulmonary arterial hypertension (PAH) is a disease of complex aetiology involving in varying degrees both genetic and environmental factors.


BACKGROUND
Thanks to progress in biology over the past 15 years the physiological consequences of cellular and molecular abnormalities are much better understood. Recent work has allowed better understanding of the different cellular signalling pathways controlling pulmonary vascular tone and cell growth. It appears that these pathways form a dense and complex network involving several groups of molecules of which NO, cGMP, ET-1 and its receptors, are at the most important.


CONCLUSIONS
The pathophysiology of PAH may be regarded as a disorder of cellular signaling where molecular abnormalities disturb the balance between the different factors controlling vascular tone and cell proliferation.


CONCLUSIONS
PAH may be viewed as a disease of cellular signalling where the molecular abnormalities not only affect a single signalling pathway but involve multiple cross-talks between groups of molecules controlling vascular smooth muscle tone and cell growth and differentiation.
Dao
P
P
Service de Physiologie-Explorations Fonctionnelles, Hôpital Cochin, AP-HP, UPRES-EA 2511, Faculté de Médecine, Université Paris-Descartes, France.
Montani
D
D
Duong-Quy
S
S
Dinh-Xuan
A T
AT
D015106:3',5'-Cyclic-GMP Phosphodiesterases; D000818:Animals; D006152:Cyclic GMP / Q000502:physiology; D054706:Cyclic Nucleotide Phosphodiesterases, Type 5; D016232:Endothelins / Q000502:physiology; D006162:Guanylate Cyclase / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology*; D009569:Nitric Oxide / Q000502:physiology; D010727:Phosphoric Diester Hydrolases / Q000502:physiology; D015398:Signal Transduction
D016232:Endothelins; D009569:Nitric Oxide; D010727:Phosphoric Diester Hydrolases; D015106:3',5'-Cyclic-GMP Phosphodiesterases; D054706:Cyclic Nucleotide Phosphodiesterases, Type 5; C517381:PDE5A protein, human; D006162:Guanylate Cyclase; D006152:Cyclic GMP