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Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2016,11,15]]},"abstract":"<jats:title>Significance<\/jats:title>\n                  <jats:p>\n                    We have found that long telomeres protect mice from genetic cardiac diseases analogous to those found in humans, such as Duchenne muscular dystrophy (DMD). Mice lacking dystrophin, similar to patients with DMD, exhibit only mild disease. In contrast, mice that lack dystrophin and have \u201chumanized\u201d telomere lengths (\n                    <jats:italic>mdx<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>4cv<\/jats:italic>\n                    <\/jats:sup>\n                    <jats:italic>\/mTR<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>G2<\/jats:italic>\n                    <\/jats:sup>\n                    ) fully manifest both the severe human skeletal muscle wasting and cardiac failure typical of DMD. Remarkably, telomere shortening accompanies cardiac development even after cardiomyocyte division has ceased. This chronic proliferation-independent shortening in dystrophin-deficient cardiomyocytes is associated with induction of a DNA damage response, mitochondrial dysfunction, increased oxidative stress, and metabolic failure. Our findings highlight an interplay between telomere length and mitochondrial homeostasis in the etiology of dystrophic heart failure.\n                  <\/jats:p>","DOI":"10.1073\/pnas.1615340113","type":"journal-article","created":{"date-parts":[[2016,10,31]],"date-time":"2016-10-31T21:59:38Z","timestamp":1477951178000},"page":"13120-13125","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":81,"title":["Telomere shortening and metabolic compromise underlie dystrophic cardiomyopathy"],"prefix":"10.1073","volume":"113","author":[{"given":"Alex Chia Yu","family":"Chang","sequence":"first","affiliation":[{"name":"Baxter Laboratory for Stem Cell Biology, Stanford University School of Medicine, Stanford University, Stanford, CA 94305;"},{"name":"Department of Microbiology and Immunology, Stanford University School of Medicine, Stanford University, Stanford, CA 94305;"},{"name":"Institute for Stem Cell Biology and Regenerative Medicine, Stanford University School of Medicine, Stanford University, Stanford, CA 94305;"},{"name":"Stanford Cardiovascular Institute, Stanford University School of Medicine, Stanford University, Stanford, CA 94305;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sang-Ging","family":"Ong","sequence":"additional","affiliation":[{"name":"Stanford Cardiovascular Institute, Stanford University School of Medicine, Stanford University, Stanford, CA 94305;"},{"name":"Division of Cardiology, Department of Medicine Stanford and Department of Radiology, Stanford University School of Medicine, Stanford University, Stanford, CA 94305;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edward L.","family":"LaGory","sequence":"additional","affiliation":[{"name":"Division of Radiation and Cancer Biology and Center for Clinical Sciences Research, Department of Radiation Oncology, Stanford University, Stanford, CA 94305"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peggy E.","family":"Kraft","sequence":"additional","affiliation":[{"name":"Baxter Laboratory for Stem Cell Biology, Stanford University School of Medicine, Stanford University, Stanford, CA 94305;"},{"name":"Department of Microbiology and Immunology, Stanford University School of Medicine, Stanford University, Stanford, CA 94305;"},{"name":"Institute for Stem Cell Biology and Regenerative Medicine, Stanford University School of Medicine, Stanford University, Stanford, CA 94305;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amato J.","family":"Giaccia","sequence":"additional","affiliation":[{"name":"Division of Radiation and Cancer Biology and Center for Clinical Sciences Research, Department of Radiation Oncology, Stanford University, Stanford, CA 94305"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph C.","family":"Wu","sequence":"additional","affiliation":[{"name":"Stanford Cardiovascular Institute, Stanford University School of Medicine, Stanford University, Stanford, CA 94305;"},{"name":"Division of Cardiology, Department of Medicine Stanford and Department of Radiology, Stanford University School of Medicine, Stanford University, Stanford, CA 94305;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helen M.","family":"Blau","sequence":"additional","affiliation":[{"name":"Baxter Laboratory for Stem Cell Biology, Stanford University School of Medicine, Stanford University, Stanford, CA 94305;"},{"name":"Department of Microbiology and Immunology, Stanford University School of Medicine, Stanford University, Stanford, CA 94305;"},{"name":"Institute for Stem Cell Biology and Regenerative Medicine, Stanford University School of Medicine, Stanford University, Stanford, CA 94305;"},{"name":"Stanford Cardiovascular Institute, Stanford University School of Medicine, Stanford University, Stanford, CA 94305;"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[2016,10,31]]},"reference":[{"key":"e_1_3_3_1_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(87)90504-6"},{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0095-5108(18)30549-9"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.3659917"},{"key":"e_1_3_3_4_2","first-page":"499","article-title":"Early muscular dystrophy: Differential patterns of weakness in Duchenne, limb-girdle and facioscapulohumeral types","volume":"47","author":"Chyatte SB","year":"1966","unstructured":"SB Chyatte, Jr PJJ Vignos, M Watkins, Early muscular dystrophy: Differential patterns of weakness in Duchenne, limb-girdle and facioscapulohumeral types. 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