{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T04:48:24Z","timestamp":1761540504960},"reference-count":0,"publisher":"Cambridge University Press (CUP)","issue":"6","license":[{"start":{"date-parts":[[2004,3,8]],"date-time":"2004-03-08T00:00:00Z","timestamp":1078704000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Expert Rev. Mol. Med."],"published-print":{"date-parts":[[2004,3,9]]},"abstract":"<jats:p>Primary pulmonary hypertension (PPH) is defined clinically by sustained elevation of pulmonary arterial pressure without a demonstrable cause, and is a progressive, often-fatal disease. PPH can be associated with ingestion of appetite suppressants, human immunodeficiency virus infection and certain autoimmune diseases. Familial PPH is known to account for 6% of all cases. Mutations in the gene encoding the bone morphogenetic protein (BMP) type II receptor have been identified in 72% of affected families and 26% of apparently sporadic cases. BMPs are members of the transforming growth factor \u03b2 superfamily and affect intracellular signalling via Smads and mitogen-activated protein kinases. Evidence supports a \u2018two-hit\u2019 hypothesis in which PPH is triggered by accumulation of genetic and environmental insults in a susceptible individual. Elucidation of the precise molecular and cellular mechanisms underlying PPH will provide a powerful basis for the development of novel therapeutic strategies in the treatment of this devastating condition.<\/jats:p>","DOI":"10.1017\/s1462399404007483","type":"journal-article","created":{"date-parts":[[2004,3,9]],"date-time":"2004-03-09T12:38:28Z","timestamp":1078835908000},"page":"1-15","source":"Crossref","is-referenced-by-count":4,"title":["Primary pulmonary hypertension: molecular basis and potential for therapy"],"prefix":"10.1017","volume":"6","author":[{"given":"Nung","family":"Rudarakanchana","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicholas W.","family":"Morrell","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"56","published-online":{"date-parts":[[2004,3,8]]},"container-title":["Expert Reviews in Molecular Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S1462399404007483","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,3,30]],"date-time":"2019-03-30T18:54:32Z","timestamp":1553972072000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S1462399404007483\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,3,8]]},"references-count":0,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2004,3,9]]}},"alternative-id":["S1462399404007483"],"URL":"https:\/\/doi.org\/10.1017\/s1462399404007483","relation":{},"ISSN":["1462-3994"],"issn-type":[{"value":"1462-3994","type":"electronic"}],"subject":[],"published":{"date-parts":[[2004,3,8]]}}}