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Lupus, Vol. 12, No. 3, 209-212 (2003)
DOI: 10.1191/0961203303lu359xx

Genetic studies of pulmonary arterial hypertension

J H Morse

Columbia University College of Physicians and Surgeons, Department of Medicine, New York, US, jhm4{at}columbia.edu

These genetic studies of primary pulmonary hypertension (PPH) initially tried to define immunogenetic subsets. Because only small subsets could be classified when defined by HLA=autoantibodyassociationsand the familial form of PPH failed to segregate with the HLA class II locus, the focus shifted to a genome scan of families with PPH (FPPH). This approach identified a gene on chromosome 2q33,34 called PPH1. Mutations in this gene, now known to be bone morphogenetic protein receptor 2 (BMPR2), can cause PPH. Mutations in a second gene, ALK-1, present in families with hereditary hemorrhagic telangiectasia type 2, also causes PPH. Both genes, involved in TGF-B signaling, provide exciting clues for defining the pathogenesis of PPH.

Key Words: bone morphogenetic protein receptor 2 • primary pulmonary hypertension • appetite suppressant PPH • pulmonary arterial hypertension • activin-like receptor 1 • autoimmune pulmonary hypertension


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