Aug 7, 2018

Recent advances in pulmonary arterial hypertension

F1000Research
Martin R WilkinsChristopher J Rhodes

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.

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Mentioned in this Paper

Pulmonary Arterial Hypertension
Bone morphogenetic protein receptor type II
Research
Genes
Polycyclic Hydrocarbons, Aromatic
Right Ventricular Failure
Idiopathic Pulmonary Hypertension
Proteomics
Pharmacologic Substance
Heart Failure, Right-Sided

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