{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T03:46:22Z","timestamp":1791431182617,"version":"4.3.3"},"reference-count":37,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"5","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2012,9,26]]},"abstract":"<jats:sec xml:lang=\"en\">\n                    <jats:title>Background<\/jats:title>\n                    <jats:p xml:lang=\"en\">\n                      Mitochondrial fusion protein mutations are a cause of inherited neuropathies such as Charcot\u2013Marie\u2013Tooth disease and dominant optic atrophy. Previously we reported that the fusion protein optic atrophy 1 (\n                      <jats:styled-content style=\"fixed-case\">OPA<\/jats:styled-content>\n                      1) is decreased in heart failure.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Methods and Results<\/jats:title>\n                    <jats:p xml:lang=\"en\">\n                      We investigated cardiac function, mitochondrial function, and\n                      <jats:styled-content style=\"fixed-case\">mtDNA<\/jats:styled-content>\n                      stability in a mouse model of the disease with\n                      <jats:styled-content style=\"fixed-case\">OPA<\/jats:styled-content>\n                      1 mutation. The homozygous mutation is embryonic lethal. Heterozygous\n                      <jats:styled-content style=\"fixed-case\">OPA<\/jats:styled-content>\n                      <jats:sup>+\/\u2212<\/jats:sup>\n                      mice exhibit reduced\n                      <jats:styled-content style=\"fixed-case\">mtDNA<\/jats:styled-content>\n                      copy number and decreased expression of nuclear antioxidant genes at 3 to 4 months. Although initial cardiac function was normal, at 12 months the\n                      <jats:styled-content style=\"fixed-case\">OPA<\/jats:styled-content>\n                      1\n                      <jats:sup>+\/\u2212<\/jats:sup>\n                      mouse hearts had decreased fractional shortening, cardiac output, and myocyte contraction. This coincided with the onset of blindness. In addition to small fragmented mitochondria, aged\n                      <jats:styled-content style=\"fixed-case\">OPA<\/jats:styled-content>\n                      1\n                      <jats:sup>+\/\u2212<\/jats:sup>\n                      mice had impaired cardiac mitochondrial function compared with wild\u2010type littermates.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p xml:lang=\"en\">\n                      <jats:styled-content style=\"fixed-case\">OPA<\/jats:styled-content>\n                      1 mutation leads to deficiency in antioxidant transcripts, increased reactive oxygen species, mitochondrial dysfunction, and late\u2010onset cardiomyopathy.\n                    <\/jats:p>\n                  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