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Glucose\u20106\u2010phosphatase deficiency, now termed type 1 glycogen storage disease, was first described almost 40 years ago but until recently very little was known about the molecular basis of the various type 1 glycogen storage diseases. Recently we have shown that at least six different proteins are needed for normal glucose\u20106\u2010phosphatase activity in liver. Four of the proteins have been purified and three cloned. Study of the type 1 glycogen storage diseases has stimulated investigations of the mechanisms of small molecule transport across the endoplasmic reticulum membrane and demonstrated the existence of novel endoplasmic reticulum transport proteins for glucose and phosphate.<\/jats:p>","DOI":"10.1002\/bies.950140609","type":"journal-article","created":{"date-parts":[[2005,2,25]],"date-time":"2005-02-25T10:19:58Z","timestamp":1109326798000},"page":"395-400","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":48,"title":["The molecular basis of the type 1 glycogen storage diseases"],"prefix":"10.1002","volume":"14","author":[{"given":"Ann","family":"Burchell","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,2,5]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/0968-0004(85)90236-1"},{"key":"e_1_2_1_3_2","first-page":"91","volume-title":"Vitamins and Hormones","author":"Ashmore J.","year":"1959"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1096\/fasebj.4.12.2168325"},{"key":"e_1_2_1_5_2","first-page":"93","volume-title":"Genetics and Human Nutrition","author":"Burchell A.","year":"1990"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/0167-4889(91)90146-O"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(18)38504-1"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF02171563"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198401193100306"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199008303230907"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(87)90484-3"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(89)90484-4"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/0304-4165(91)90197-O"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1042\/bj2550471"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1210\/endo-126-5-2413"},{"key":"e_1_2_1_16_2","doi-asserted-by":"crossref","first-page":"9732","DOI":"10.1016\/S0021-9258(18)81716-1","article-title":"Evidence for the presence of glucose cycling in pancreatic islets of the ob\/ob mouse","volume":"264","author":"Khan A.","year":"1989","journal-title":"J. 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