Pathobiology of pulmonary arterial hypertension: understanding the roads less travelled

被引:75
作者
Hemnes, Anna R. [1 ]
Humbert, Marc [2 ,3 ]
机构
[1] Vanderbilt Univ, Med Ctr, Dept Med, 1161 21st Ave South, Nashville, TN 37232 USA
[2] Univ Paris Sud, Univ Paris Saclay, Fac Med, Le Kremlin Bicetre, France
[3] Hop Bicetre, AP HP, Ctr Reference Hypertens Pulmonaire, Serv Pneumol, Le Kremlin Bicetre, France
关键词
TO-MESENCHYMAL TRANSITION; FIBROBLAST GROWTH FACTOR-2; IRON-DEFICIENCY; ENDOTHELIAL DYSFUNCTION; POTASSIUM CHANNELS; IMATINIB MESYLATE; FACTOR EXPRESSION; II RECEPTOR; DNA-DAMAGE; SURVIVAL;
D O I
10.1183/16000617.0093-2017
中图分类号
R56 [呼吸系及胸部疾病];
学科分类号
摘要
The pathobiology of pulmonary arterial hypertension (PAH) is complex and incompletely understood. Although three pathogenic pathways have been relatively well characterised, it is widely accepted that dysfunction in a multitude of other cellular processes is likely to play a critical role in driving the development of PAH. Currently available therapies, which all target one of the three well-characterised pathways, provide significant benefits for patients; however, PAH remains a progressive and ultimately fatal disease. The development of drugs to target alternative pathogenic pathways is, therefore, an attractive proposition and one that may complement existing treatment regimens to improve outcomes for patients. Considerable research has been undertaken to identify the role of the less well-understood pathways and in this review we will highlight some of the key discoveries and the potential for utility as therapeutic targets.
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页数:11
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