Molecular biology of primary pulmonary hypertension.
Primary pulmonary hypertension (PPH) is a rare but often fatal condition characterized by pulmonary artery remodeling leading to chronic elevation of pulmonary artery pressure in the absence of causes. The pathophysiology of PPH is not completely understood, but a number of recent studies have elucidated many possible gentic, hormonal, and environmental factors. Current treatment options slow the progression of the disease but do not halt it. The study of molecular mechanisms that result from mutations in onmental and hormonal modifiers holds great promise for the development of novel therapies that may halt the progression of the disease.
Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of California, San Diego, 9500 Gilman Drive, LaJolla, CA 92093-0725, USA.
Mehran
M
Mandegar
Patricia A
PA
Thistlethwaite
Jason X J
JX
Yuan
D052006:Bone Morphogenetic Protein Receptors, Type II; D002118:Calcium / Q000378:metabolism; D003251:Constriction, Pathologic; D006801:Humans; D006976:Hypertension, Pulmonary / Q000235:genetics* / Q000378:metabolism* / Q000473:pathology; D009154:Mutation; D017346:Protein Serine-Threonine Kinases / Q000235:genetics; D011956:Receptors, Cell Surface / Q000235:genetics
D011956:Receptors, Cell Surface; D017346:Protein Serine-Threonine Kinases; C498218:BMPR2 protein, human; D052006:Bone Morphogenetic Protein Receptors, Type II; D002118:Calcium