{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,2]],"date-time":"2025-11-02T16:19:51Z","timestamp":1762100391587},"reference-count":34,"publisher":"Portland Press Ltd.","issue":"1","content-domain":{"domain":["portlandpress.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2005,7,1]]},"abstract":"<jats:p>Glucose 6-phosphate transport has been well characterized in liver microsomes. The transport is required for the functioning of the glucose-6-phosphatase enzyme that is situated in the lumen of the hepatic endoplasmic reticulum. The genetic deficiency of the glucose 6-phosphate transport activity causes a severe metabolic disease termed type\u00a01b glycogen storage disease. The cDNA encoding a liver transporter for glucose 6-phosphate was cloned and was found to be mutated in patients suffering from glycogen storage disease 1b. While related mRNAs have been described in liver and other tissues, the encoded protein(s) has not been immunologically characterized yet. In the present study, we report (using antibodies against three different peptides of the predicted amino acid sequence) that a major protein encoded by the glucose 6-phosphate transporter gene is expressed in the endoplasmic reticulum membranes of rat and human liver. The protein has an apparent molecular mass of approx.\u00a033\u00a0kDa using SDS\/PAGE, but several lines of evidence indicate that its real molecular mass is 46\u00a0kDa, as expected. The glucose 6-phosphate transporter protein was also immunodetected in kidney microsomes, but not in microsomes derived from human fibrocytes, rat spleen and lung, and a variety of cell lines. Moreover, little or no expression of the glucose 6-phosphate transporter protein was found in liver microsomes obtained from three glycogen storage disease 1b patients, even bearing mutations that do not directly interfere with protein translation, which can be explained by a (proteasome-mediated) degradation of the mutated transporter.<\/jats:p>","DOI":"10.1042\/bj20050213","type":"journal-article","created":{"date-parts":[[2005,6,21]],"date-time":"2005-06-21T21:09:36Z","timestamp":1119388176000},"page":"57-62","update-policy":"http:\/\/dx.doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Immunodetection of the expression of microsomal proteins encoded by the glucose 6-phosphate transporter gene"],"prefix":"10.1042","volume":"389","author":[{"given":"Silvia","family":"SENESI","sequence":"first","affiliation":[{"name":"Dipartimento di Fisiopatologia, Medicina Sperimentale e Sanit\u00e0 Pubblica, University of Siena, Via A. Moro 3, 53100-Siena, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paola","family":"MARCOLONGO","sequence":"additional","affiliation":[{"name":"Dipartimento di Fisiopatologia, Medicina Sperimentale e Sanit\u00e0 Pubblica, University of Siena, Via A. Moro 3, 53100-Siena, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tamas","family":"KARDON","sequence":"additional","affiliation":[{"name":"Department of Medical Chemistry, Semmelweis University, 1444-Budapest, Hungary"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giovanna","family":"BUCCI","sequence":"additional","affiliation":[{"name":"Dipartimento di Fisiopatologia, Medicina Sperimentale e Sanit\u00e0 Pubblica, University of Siena, Via A. Moro 3, 53100-Siena, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrey","family":"SUKHODUB","sequence":"additional","affiliation":[{"name":"Division of Maternal and Child Health Sciences, Ninewells Hospital and Medical School, University of Dundee, Dundee DD1 9SY, U.K."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ann","family":"BURCHELL","sequence":"additional","affiliation":[{"name":"Division of Maternal and Child Health Sciences, Ninewells Hospital and Medical School, University of Dundee, Dundee DD1 9SY, U.K."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Angelo","family":"BENEDETTI","sequence":"additional","affiliation":[{"name":"Dipartimento di Fisiopatologia, Medicina Sperimentale e Sanit\u00e0 Pubblica, University of Siena, Via A. 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