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Autophagy is robustly induced in response to starvation in order to generate nutrients and energy through the lysosomal degradation of cytoplasmic components. Constitutive, basal autophagy serves as a quality control mechanism for the elimination of aggregated proteins and worn\u2010out or damaged organelles, such as mitochondria. Research during the last decade has made it clear that malfunctioning or failure of this system is associated with a wide range of human pathologies and age\u2010related diseases. Our recent data provide strong evidence for the role of autophagy in the pathogenesis of Pompe disease, a lysosomal glycogen storage disease caused by deficiency of acid alpha\u2010glucosidase (GAA). Large pools of autophagic debris in skeletal muscle cells can be seen in both our GAA knockout model and patients with Pompe disease. In this review, we will focus on these recent data, and comment on the not so recent observations pointing to the involvement of autophagy in skeletal muscle damage in Pompe disease. Published 2012. This article is a U.S. Government work and is in the public domain in the USA.<\/jats:p>","DOI":"10.1002\/ajmg.c.31317","type":"journal-article","created":{"date-parts":[[2012,1,17]],"date-time":"2012-01-17T20:56:52Z","timestamp":1326833812000},"page":"13-21","source":"Crossref","is-referenced-by-count":101,"title":["Autophagy and mitochondria in Pompe disease: Nothing is so new as what has long been forgotten"],"prefix":"10.1002","volume":"160C","author":[{"given":"Nina","family":"Raben","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amanda","family":"Wong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evelyn","family":"Ralston","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachel","family":"Myerowitz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2012,1,17]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.273.34.21883"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1159\/000114945"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02248461"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200507002"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2007-2222"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2008-3667"},{"key":"e_1_2_8_8_1","first-page":"1200","article-title":"Localization of dystrophin and beta\u2010spectrin in vacuolar myopathies","volume":"143","author":"De Bleecker JL","year":"1993","journal-title":"Am J Pathol"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/93.3.599"},{"key":"e_1_2_8_10_1","first-page":"233","article-title":"Autophagic glycogenosis of late onset with mitochondrial abnormalities: Light and electron microscopic observations","volume":"43","author":"Engel AG","year":"1968","journal-title":"Mayo Clin Proc"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(98)00117-5"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymthe.2006.08.009"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.4161\/auto.2984"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02889849"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj0860011"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddr424"},{"key":"e_1_2_8_17_1","first-page":"101","article-title":"Correlations between histochemical and ultrastructural studies of diseased muscle","volume":"19","author":"Hudgson P","year":"1975","journal-title":"Riv Istochim Norm Patol"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199101313240517"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M110.150839"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/19.21.5720"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.4161\/auto.3678"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200412022"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2007.10.035"},{"key":"e_1_2_8_24_1","first-page":"689","article-title":"Glycogen autophagy in the liver and heart of newborn rats. 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