{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T09:13:36Z","timestamp":1790327616613,"version":"4.1.0"},"reference-count":69,"publisher":"American Society for Clinical Investigation","issue":"14","funder":[{"DOI":"10.13039\/100000050","name":"NHLBI","doi-asserted-by":"crossref","award":["1R01HL132182-01"],"award-info":[{"award-number":["1R01HL132182-01"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100000050","name":"NHLBI","doi-asserted-by":"crossref","award":["R01 HL124248"],"award-info":[{"award-number":["R01 HL124248"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100000968","name":"American Heart Association","doi-asserted-by":"crossref","award":["16GRNT30680004"],"award-info":[{"award-number":["16GRNT30680004"]}],"id":[{"id":"10.13039\/100000968","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100000968","name":"American Heart Association","doi-asserted-by":"crossref","award":["18CDA34080244"],"award-info":[{"award-number":["18CDA34080244"]}],"id":[{"id":"10.13039\/100000968","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,7,25]]},"abstract":"<jats:p>\n                    Mutations in the\n                    <jats:italic>BSCL2<\/jats:italic>\n                    gene underlie human type 2 Berardinelli-Seip congenital lipodystrophy (BSCL2) disease. Global\n                    <jats:italic>\n                      Bscl2\n                      <jats:sup>\u2013\/\u2013<\/jats:sup>\n                    <\/jats:italic>\n                    mice recapitulate human BSCL2 lipodystrophy and results in the development of insulin resistance and hypertrophic cardiomyopathy. The pathological mechanisms underlying the development of lipodystrophy and cardiomyopathy in BSCL2 are controversial. Here we report that\n                    <jats:italic>\n                      Bscl2\n                      <jats:sup>\u2013\/\u2013<\/jats:sup>\n                    <\/jats:italic>\n                    mice develop cardiac hypertrophy because of increased basal IGF1 receptor\u2013mediated (IGF1R-mediated) PI3K\/AKT signaling.\n                    <jats:italic>\n                      Bscl2\n                      <jats:sup>\u2013\/\u2013<\/jats:sup>\n                    <\/jats:italic>\n                    hearts exhibited increased adipose triglyceride lipase (ATGL) protein stability and expression causing drastic reduction of glycerolipids. Excessive fatty acid oxidation was overt in\n                    <jats:italic>\n                      Bscl2\n                      <jats:sup>\u2013\/\u2013<\/jats:sup>\n                    <\/jats:italic>\n                    hearts, partially attributing to the hyperacetylation of cardiac mitochondrial proteins. Intriguingly, pharmacological inhibition or genetic inactivation of ATGL could rescue adipocyte differentiation and lipodystrophy in\n                    <jats:italic>\n                      Bscl2\n                      <jats:sup>\u2013\/\u2013<\/jats:sup>\n                    <\/jats:italic>\n                    cells and mice. Restoring a small portion of fat mass by ATGL partial deletion in\n                    <jats:italic>\n                      Bscl2\n                      <jats:sup>\u2013\/\u2013<\/jats:sup>\n                    <\/jats:italic>\n                    mice not only reversed the systemic insulin resistance, but also ameliorated cardiac protein hyperacetylation, normalized cardiac substrate metabolism, and improved contractile function. Collectively, our study uncovers pathways underlying lipodystrophy-induced cardiac hypertrophy and metabolic remodeling and pinpoints ATGL as a downstream target of BSCL2 in regulating the development of lipodystrophy and its associated cardiomyopathy.\n                  <\/jats:p>","DOI":"10.1172\/jci.insight.129781","type":"journal-article","created":{"date-parts":[[2019,6,11]],"date-time":"2019-06-11T12:02:42Z","timestamp":1560254562000},"source":"Crossref","is-referenced-by-count":36,"title":["Targeting ATGL to rescue BSCL2 lipodystrophy and its associated cardiomyopathy"],"prefix":"10.1172","volume":"4","author":[{"given":"Hongyi","family":"Zhou","sequence":"first","affiliation":[{"name":"Department of Physiology, Medical College of Georgia at Augusta University, Augusta, Georgia, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xinnuo","family":"Lei","sequence":"additional","affiliation":[{"name":"Department of Physiology, Medical College of Georgia at 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