{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T00:47:39Z","timestamp":1787014059831,"version":"build-2736575974"},"reference-count":30,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2013,7,29]],"date-time":"2013-07-29T00:00:00Z","timestamp":1375056000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Clinical Translational Sci"],"published-print":{"date-parts":[[2013,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Prader\u2013Willi syndrome (PWS) is a genetic disorder caused by deficiency of imprinted gene expression from the paternal chromosome 15q11\u201315q13 and clinically characterized by neonatal hypotonia, short stature, cognitive impairment, hypogonadism, hyperphagia, morbid obesity, and diabetes. Previous clinical studies suggest that a defect in energy metabolism may be involved in the pathogenesis of PWS. We focused our attention on the genes associated with energy metabolism and found that there were 95 and 66 mitochondrial genes differentially expressed in PWS muscle and brain, respectively. Assessment of enzyme activities of mitochondrial oxidative phosphorylation complexes in the brain, heart, liver, and muscle were assessed. We found the enzyme activities of the cardiac mitochondrial complexes II\u202bIII were up\u2010regulated in the PWS imprinting center deletion mice compared to the wild\u2010type littermates. These studies suggest that differential gene expression, especially of the mitochondrial genes may contribute to the pathophysiology of PWS.<\/jats:p>","DOI":"10.1111\/cts.12083","type":"journal-article","created":{"date-parts":[[2013,7,29]],"date-time":"2013-07-29T15:52:16Z","timestamp":1375113136000},"page":"347-355","source":"Crossref","is-referenced-by-count":27,"title":["Differential Gene Expression Reveals Mitochondrial Dysfunction in an Imprinting Center Deletion Mouse Model of Prader\u2013Willi Syndrome"],"prefix":"10.1111","volume":"6","author":[{"given":"Puya G.","family":"Yazdi","sequence":"first","affiliation":[{"name":"Division of Genetics and Metabolism Department of Pediatrics University of California  Irvine California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hailing","family":"Su","sequence":"additional","affiliation":[{"name":"Division of Genetics and Metabolism Department of Pediatrics University of California  Irvine California 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