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Recent advances in pulmonary arterial hypertension.

ABSTRACT: Pulmonary arterial hypertension (PAH) is a rare disorder with a high mortality rate. Treatment options have improved in the last 20 years, but patients still die prematurely of right heart failure. Though rare, it is heterogeneous at the genetic and molecular level, and understanding and exploiting this is key to the development of more effective treatments. BMPR2, encoding bone morphogenetic receptor type 2, is the most commonly affected gene in both familial and non-familial PAH, but rare mutations have been identified in other genes. Transcriptomic, proteomic, and metabolomic studies looking for endophenotypes are under way. There is no shortage of candidate new drug targets for PAH, but the selection and prioritisation of these are challenges for the research community.

SUBMITTER: Wilkins MR

PROVIDER: S-EPMC6058465 | biostudies-literature | 2018

REPOSITORIES: biostudies-literature

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Recent advances in pulmonary arterial hypertension.

Wilkins Martin R MR Aman Jurjan J Harbaum Lars L Ulrich Anna A Wharton John J Rhodes Christopher J CJ

F1000Research 20180724

Pulmonary arterial hypertension (PAH) is a rare disorder with a high mortality rate. Treatment options have improved in the last 20 years, but patients still die prematurely of right heart failure. Though rare, it is heterogeneous at the genetic and molecular level, and understanding and exploiting this is key to the development of more effective treatments. <i>BMPR2</i>, encoding bone morphogenetic receptor type 2, is the most commonly affected gene in both familial and non-familial PAH, but ra ...[more]

PMID: 30079232

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