Diseases of the circulatory system

Nesterova, Anastasia P.; Klimov, Eugene; Zharkova, Maria; Sozin, Sergey; Соболев, В. В.; Ivanikova, Natalia V.; Shkrob, Maria; Yuryev, Anton

doi:10.1016/b978-0-12-817086-1.00008-7

Cited by 2 publications

(1 citation statement)

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“…PH is characterized by pulmonary artery remodeling and increased pulmonary vascular resistance, leading to right ventricular dysfunction ( Berkelhamer et al, 2013b ; Hansmann et al, 2021 ). Another feature of PH pathophysiology is pulmonary artery smooth muscle cell (PASMC) and endothelial cell hyperproliferation and resistance to apoptosis, causing pulmonary artery wall thickening and lumen narrowing ( Marshall et al, 2018 ; Humbert et al, 2019 ; Nesterova et al, 2020 ). Mitochondrial dysfunction and dysregulation are also operative in PH pathogenesis ( Trenker et al, 2007 ; Angara and Bhandari, 2013 ; Dromparis et al, 2013 ; Pak et al, 2013 ; Adesina et al, 2015 ; Bruns et al, 2015 ; Diebold et al, 2015 ; Houten, 2015 ; Chan and Rubin, 2017 ; Hong et al, 2017 ; Culley and Chan, 2018 ; Marshall et al, 2018 ; Rafikova et al, 2018 ; Suliman and Nozik-Grayck, 2019 ).…”

Section: Mitochondrial Dysfunction In Preterm Infants and Its Downstr...mentioning

confidence: 99%

PGC-1α activity and mitochondrial dysfunction in preterm infants

2022

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Extremely low gestational age neonates (ELGANs) are born in a relatively hyperoxic environment with weak antioxidant defenses, placing them at high risk for mitochondrial dysfunction affecting multiple organ systems including the nervous, respiratory, ocular, and gastrointestinal systems. The brain and lungs are highly affected by mitochondrial dysfunction and dysregulation in the neonate, causing white matter injury (WMI) and bronchopulmonary dysplasia (BPD), respectively. Adequate mitochondrial function is important in providing sufficient energy for organ development as it relates to alveolarization and axonal myelination and decreasing oxidative stress via reactive oxygen species (ROS) and reactive nitrogen species (RNS) detoxification. Peroxisome proliferator-activated receptor gamma coactivator-1 alpha (PGC-1α) is a master regulator of mitochondrial biogenesis and function. Since mitochondrial dysfunction is at the root of WMI and BPD pathobiology, exploring therapies that can regulate PGC-1α activity may be beneficial. This review article describes several promising therapeutic agents that can mitigate mitochondrial dysfunction through direct and indirect activation and upregulation of the PGC-1α pathway. Metformin, resveratrol, omega 3 fatty acids, montelukast, L-citrulline, and adiponectin are promising candidates that require further pre-clinical and clinical studies to understand their efficacy in decreasing the burden of disease from WMI and BPD in preterm infants.

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