{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T06:02:50Z","timestamp":1791180170111,"version":"4.1.0"},"reference-count":55,"publisher":"Oxford University Press (OUP)","issue":"8","license":[{"start":{"date-parts":[[2013,4,1]],"date-time":"2013-04-01T00:00:00Z","timestamp":1364774400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2013,4,15]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Rationale<\/jats:title>\n                    <jats:p>Pulmonary arterial hypertension (PAH) is a lethal, female-predominant, vascular disease. Pathologic changes in PA smooth muscle cells (PASMC) include excessive proliferation, apoptosis-resistance, and mitochondrial fragmentation. Activation of dynamin-related protein increases mitotic fission and promotes this proliferation\u2013apoptosis imbalance. The contribution of decreased fusion and reduced mitofusin-2 (MFN2) expression to PAH is unknown.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>We hypothesize that decreased MFN2 expression promotes mitochondrial fragmentation, increases proliferation, and impairs apoptosis. The role of MFN2\u2019s transcriptional coactivator, peroxisome proliferator-activated receptor \u03b3 coactivator 1-\u03b1 (PGC1\u03b1), was assessed. MFN2 therapy was tested in PAH PASMC and in models of PAH.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Fusion and fission mediators were measured in lungs and PASMC from patients with PAH and female rats with monocrotaline or chronic hypoxia+Sugen-5416 (CH+SU) PAH. The effects of adenoviral mitofusin-2 (Ad-MFN2) overexpression were measured in vitro and in vivo.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Measurements and Main Results<\/jats:title>\n                    <jats:p>In normal PASMC, siMFN2 reduced expression of MFN2 and PGC1\u03b1; conversely, siPGC1\u03b1 reduced PGC1\u03b1 and MFN2 expression. Both interventions caused mitochondrial fragmentation. siMFN2 increased proliferation. In rodent and human PAH PASMC, MFN2 and PGC1\u03b1 were decreased and mitochondria were fragmented. Ad-MFN2 increased fusion, reduced proliferation, and increased apoptosis in human PAH and CH+SU. In CH+SU, Ad-MFN2 improved walking distance (381 \u00b1 35 vs. 245 \u00b1 39 m; P &amp;lt; 0.05); decreased pulmonary vascular resistance (0.18 \u00b1 0.02 vs. 0.38 \u00b1 0.14 mm Hg\/ml\/min; P &amp;lt; 0.05); and decreased PA medial thickness (14.5 \u00b1 0.8 vs. 19 \u00b1 1.7%; P &amp;lt; 0.05). Lung vascularity was increased by MFN2.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Decreased expression of MFN2 and PGC1\u03b1 contribute to mitochondrial fragmentation and a proliferation\u2013apoptosis imbalance in human and experimental PAH. Augmenting MFN2 has therapeutic benefit in human and experimental PAH.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1164\/rccm.201209-1687oc","type":"journal-article","created":{"date-parts":[[2013,3,1]],"date-time":"2013-03-01T14:42:47Z","timestamp":1362148967000},"page":"865-878","source":"Crossref","is-referenced-by-count":184,"title":["PGC1\u03b1-mediated Mitofusin-2 Deficiency in Female Rats and Humans with Pulmonary Arterial Hypertension"],"prefix":"10.1093","volume":"187","author":[{"given":"John J.","family":"Ryan","sequence":"first","affiliation":[{"name":"Section of Cardiology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Glenn","family":"Marsboom","sequence":"additional","affiliation":[{"name":"Section of Cardiology"},{"name":"Immunology Department, University Hospital La Princesa, Automomous University, Madrid, Spain; 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