Recent advances in pulmonary arterial hypertension.

F1000Res 2018 24;7. Epub 2018 Jul 24.

Department of Medicine, Imperial College London, London, UK.

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. , 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.

Download full-text PDF

Source
http://dx.doi.org/10.12688/f1000research.14984.1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6058465PMC
July 2018
6 Reads

Publication Analysis

Top Keywords

pah rare
8
arterial hypertension
8
pulmonary arterial
8
level understanding
4
molecular level
4
metabolomic studies
4
exploiting key
4
key development
4
proteomic metabolomic
4
understanding exploiting
4
genetic molecular
4
non-familial pah
4
heart failure
4
shortage candidate
4
rare heterogeneous
4
endophenotypes shortage
4
development effective
4
heterogeneous genetic
4
studies endophenotypes
4
effective treatments
4

References

(Supplied by CrossRef)
A global view of pulmonary hypertension.
M Hoeper et al.
Lancet Respir Med. 2016

Similar Publications