The DOI matches its Crossref record; 61 of the 61 references it cites were found in a registry or an index.
Sirtuin 3 Deficiency Is Associated with Inhibited Mitochondrial Function and Pulmonary Arterial Hypertension in Rodents and Humans
Open accessAbstract
Suppression of mitochondrial function promoting proliferation and apoptosis suppression has been described in the pulmonary arteries and extrapulmonary tissues in pulmonary arterial hypertension (PAH), but the cause of this metabolic remodeling is unknown. Mice lacking sirtuin 3 (SIRT3), a mitochondrial deacetylase, have increased acetylation and inhibition of many mitochondrial enzymes and complexes, suppressing mitochondrial function. Sirt3KO mice develop spontaneous PAH, exhibiting previously described molecular features of PAH pulmonary artery smooth muscle cells (PASMC). In human PAH PASMC and rats with PAH, SIRT3 is downregulated, and its normalization with adenovirus gene therapy reverses the disease phenotype. A loss-of-function SIRT3 polymorphism, linked to metabolic syndrome, is associated with PAH in an unbiased cohort of 162 patients and controls. If confirmed in large patient cohorts, these findings may facilitate biomarker and therapeutic discovery programs in PAH.
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“Increased acetylation of mitochondrial proteins in the affected smooth muscle leads to mitochondrial hyperpolarization, increased proliferation, suppressed apoptosis, and eventually to progressive arterial thickening.”
From The Mitochondrial Acylome Emerges: Proteomics, Regulation by Sirtuins, and Metabolic and Disease Implications (2018) · cites this paper as Paulin et al. 2014
In the citing paper
“Recent work by the Michelakis’s group has identified loss of SIRT3 function as a potential cause of this disease ((Paulin et al. 2014))”
From The Mitochondrial Acylome Emerges: Proteomics, Regulation by Sirtuins, and Metabolic and Disease Implications (2018) · cites this paper as Paulin et al. 2014
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Sirtuin 3 is required for pro-inflammatory and pro-regenerative aspects of senescent cellsPreprint
Wiley, C. D., Mogck, B. A., Jezak, S. T., & Velarde, M. C. (2024) · Research Square · doi:10.21203/rs.3.rs-4656269/v1
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Adventitial fibroblasts direct smooth muscle cell-state transition in pulmonary vascular diseasePreprint
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Pulmonary Arterial Hypertension: Reconfiguring the Vascular Landscape to Reverse Remodeling
Vassiliou, A. G., Papavassiliou, K. A., Lotsios, N. S., Orfanos, S. E., & Papavassiliou, A. G. (2026) · Cells · doi:10.3390/cells15040332
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Loss of sirtuin 3 disrupts cellular senescence signaling pathways
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Metadata
- Authors
- Paulin, R., Dromparis, P., Sutendra, G., Gurtu, V., Zervopoulos, S., Bowers, L., Haromy, A., Webster, L., Provencher, S., Bonnet, S., & Michelakis, E. D.
- Affiliations
- University of Alberta (CA)
- Institut universitaire de cardiologie et de pneumologie de Québec (CA)
- Corresponding author
- Michelakis, Evangelos D.
- ORCID
- Venue
- Cell Metabolism
- Publisher
- Elsevier BV
- Published
- 2014-11
- Volume
- 20
- Issue
- 5
- Pages
- 827-839
- ISSN
- 1550-4131 (ISSN-L)
- OpenAlex
- W2153420197
- Cited by
- 214
- References
- 61
- Citations by year
- 2026: 9 · 2025: 12 · 2024: 19 · 2023: 16 · 2022: 19 · 2021: 12 · 2020: 19 · 2019: 19 · 2018: 25 · 2017: 21 · 2016: 26 · 2015: 13 · 2014: 1
- FWCI
- 16.21
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Cell Metabolism (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Canadian Institutes of Health Research
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Health Sciences › Medicine › Geriatrics and Gerontology › Sirtuins and Resveratrol in Medicine
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Keywords
- SIRT3, pulmonary arterial hypertension, mitochondrial function, mitochondrial deacetylase, pulmonary artery smooth muscle cells, SIRT3 knockout mice, adenoviral gene therapy, metabolic syndrome, biomarkers
- MeSH terms
- Adult, Animals, Cells, Cultured, Female, Humans, Hypertension, Pulmonary, Lung, Middle Aged, Mitochondria, Polymorphism, Genetic, Pulmonary Artery, Genetic Therapy, Down-Regulation, Rats, Sprague-Dawley, Mice, Knockout, Mice, Rats, Sirtuin 3, Familial Primary Pulmonary Hypertension
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