Related Experiment Video
Updated: Jun 5, 2025
08:02
Isolation of Pulmonary Artery Smooth Muscle Cells from Neonatal Mice
Published on: October 19, 2013
18.3K
Alterations in Mitochondrial Function in Pulmonary Vascular Diseases
Samar Farha1,2, Kewal Asosingh1, Paul M Hassoun3
1Integrated Hospital-Care Institute, Cleveland Clinic, Cleveland, Ohio, USA.
Antioxidants & Redox Signaling
|December 10, 2024
Summary
Mitochondrial dysfunction and altered arginine metabolism are common in pulmonary hypertension (PH). Group 1 PAH patients show a unique mitochondrial phenotype linked to fatty acid metabolism and survival.
Area of Science:
- Mitochondrial biology
- Cardiovascular research
- Metabolomics
Background:
- Pulmonary arterial hypertension (PAH) involves mitochondrial and arginine metabolism alterations.
- Limited understanding exists regarding mitochondrial function across diverse pulmonary hypertension (PH) groups.
Purpose of the Study:
- To investigate mitochondrial function and arginine metabolism across all PH groups.
- To associate these alterations with clinical phenotypes and survival.
Main Methods:
- Analysis of platelet mitochondrial transmembrane potential, superoxide production, and mass via flow cytometry.
- Plasma metabolomics and calculation of global arginine bioavailability.
- Clinical phenotyping and 4-year follow-up for death or transplant in the Pulmonary Vascular Disease Phenomics Program cohort.
Main Results:
- All PH groups exhibited lower global arginine bioavailability than controls.
- Mitochondrial superoxide was elevated in group 1 PAH and lowest in group 3.
- Group 1 PAH showed reduced mitochondrial transmembrane potential, linked to fatty acid metabolism and transplant-free survival.
Conclusions:
- Mitochondrial dysfunction is present in all PH groups.
- Group 1 PAH displays a distinct mitochondrial phenotype characterized by increased superoxide and decreased transmembrane potential.
- These findings in group 1 PAH are associated with fatty acid metabolism and clinical outcomes.
Keywords:
arginine metabolismmitochondriapulmonary hypertensionsuperoxide productiontransmembrane potentialMore Related Videos
09:40Phosphorus-31 Magnetic Resonance Spectroscopy: A Tool for Measuring In Vivo Mitochondrial Oxidative Phosphorylation Capacity in Human Skeletal Muscle
Published on: January 19, 2017
11.7K
08:12Author Spotlight: Unveiling Mitochondrial Function and Cellular Metabolic Adaptation in Metabolic Diseases
Published on: October 4, 2024
1.5K
Related Concept Videos
01:20Chronic Obstructive Pulmonary Disease-II: Pathophysiology
2.7K
Chronic Obstructive Pulmonary Disease (COPD) pathophysiology is intricate and multifaceted, involving a complex interplay of physiological processes. Understanding these mechanisms is crucial for effectively managing and treating COPD. Here is an in-depth look at the critical elements in the pathophysiology of COPD:
Chronic Inflammation
Chronic Inflammation
2.7K
01:30Pulmonary Hypertension: Classification and Pathogenesis
150
Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
There are various classifications for PH, each relating to different underlying causes and also...
There are various classifications for PH, each relating to different underlying causes and also...
150
01:22Mitral Stenosis I: Introduction
2
Mitral Valve Stenosis (MVS) is a heart condition where the mitral valve narrows, impeding blood circulation from the left atrium to the left ventricle. The etiology and pathophysiology of this condition are multifaceted, leading to a cascade of cardiovascular complications.Causes of Mitral Valve StenosisRheumatic Heart Disease: It is the main cause of mitral valve stenosis, particularly in developing nations. This condition arises from rheumatic fever, an inflammatory illness resulting from...
2
01:46Electron Transport Chain: Complex I and II
11.5K
The mitochondrial electron transport chain (ETC) is the main energy generation system in the eukaryotic cells. However, mitochondria also produce cytotoxic reactive oxygen species (ROS) due to the large electron flow during oxidative phosphorylation. While Complex I is one of the primary sources of superoxide radicals, ROS production by Complex II is uncommon and may only be observed in cancer cells with mutated complexes.
ROS generation is regulated and maintained at moderate levels necessary...
ROS generation is regulated and maintained at moderate levels necessary...
11.5K
01:37Mitochondria
10.9K
Mitochondria are eukaryotic cellular organelles that are known to produce energy through a process called oxidative phosphorylation. Besides their primary function, mitochondria are involved in various cellular processes, including cell growth, differentiation, signaling, metabolism, and senescence. Age-related changes cause a decline in mitochondrial quality and integrity due to increased mitochondrial mutations and oxidative damage. Thus, aging can severely impact mitochondrial functions,...
10.9K
01:45Mitochondrial Membranes
8.6K
A single mitochondrion is a bean-shaped organelle enclosed by a double-membrane system. The outer membrane of mitochondria is smooth and contains many porins - the integral membrane transporters. Porins enable free diffusion of ions and small uncharged molecules through the outer mitochondrial membrane but limit the transport of molecules larger than 5000 Daltons. Further, the outer mitochondrial membrane forms a unique structure called membrane contact sites with other subcellular organelles,...
8.6K