| Autor: |
Xu W; Lerner Research Institute, Cleveland Clinic, Cleveland, Ohio 44195, USA; email: erzurus@ccf.org., Janocha AJ; Lerner Research Institute, Cleveland Clinic, Cleveland, Ohio 44195, USA; email: erzurus@ccf.org., Erzurum SC; Lerner Research Institute, Cleveland Clinic, Cleveland, Ohio 44195, USA; email: erzurus@ccf.org.; Respiratory Institute, Cleveland Clinic, Cleveland, Ohio 44195, USA. |
| Jazyk: |
angličtina |
| Zdroj: |
Annual review of physiology [Annu Rev Physiol] 2021 Feb 10; Vol. 83, pp. 551-576. |
| DOI: |
10.1146/annurev-physiol-031620-123956 |
| Abstrakt: |
Pulmonary arterial hypertension (PAH) is characterized by impaired regulation of pulmonary hemodynamics and vascular growth. Alterations of metabolism and bioenergetics are increasingly recognized as universal hallmarks of PAH, as metabolic abnormalities are identified in lungs and hearts of patients, animal models of the disease, and cells derived from lungs of patients. Mitochondria are the primary organelle critically mediating the complex and integrative metabolic pathways in bioenergetics, biosynthetic pathways, and cell signaling. Here, we review the alterations in metabolic pathways that are linked to the pathologic vascular phenotype of PAH, including abnormalities in glycolysis and glucose oxidation, fatty acid oxidation, glutaminolysis, arginine metabolism, one-carbon metabolism, the reducing and oxidizing cell environment, and the tricarboxylic acid cycle, as well as the effects of PAH-associated nuclear and mitochondrial mutations on metabolism. Understanding of the metabolic mechanisms underlying PAH provides important knowledge for the design of new therapeutics for treatment of patients. |
| Databáze: |
MEDLINE |
| Externí odkaz: |
|
