{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T11:47:08Z","timestamp":1791978428960,"version":"4.4.0"},"reference-count":46,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2008,3,12]],"date-time":"2008-03-12T00:00:00Z","timestamp":1205280000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["faseb.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The FASEB Journal"],"published-print":{"date-parts":[[2008,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Glycogen storage disease type Ib (GSD\u2010Ib) is caused by deficiencies in the glucose\u20106\u2010phosphate (G6P) transporter (G6PT) that have been well characterized. Interestingly, deleterious mutations in the\n                    <jats:italic>G6PT<\/jats:italic>\n                    gene were identified in clinical cases of GSD type Ic (GSD\u2010Ic) proposed to be deficient in an inorganic phosphate (P\n                    <jats:sub>i<\/jats:sub>\n                    ) transporter. We hypothesized that G6PT is both the G6P and P\n                    <jats:sub>i<\/jats:sub>\n                    transporter. Using reconstituted proteoliposomes we show that both G6P and P\n                    <jats:sub>i<\/jats:sub>\n                    are efficiently taken up into P\n                    <jats:sub>i<\/jats:sub>\n                    \u2010loaded G6PT\u2010proteoliposomes. The G6P uptake activity decreases as the internal:external P\n                    <jats:sub>i<\/jats:sub>\n                    ratio decreases and the P\n                    <jats:sub>i<\/jats:sub>\n                    uptake activity decreases in the presence of external G6P. Moreover, G6P or P\n                    <jats:sub>i<\/jats:sub>\n                    uptake activity is not detectable in P\n                    <jats:sub>i<\/jats:sub>\n                    \u2010loaded proteoliposomes containing the p.R28H G6PT null mutant. The G6PT\u2010proteoliposome\u2010 mediated G6P or P\n                    <jats:sub>i<\/jats:sub>\n                    uptake is inhibited by cholorgenic acid and vanadate, both specific G6PT inhibitors. Glucose\u20106\u2010phosphatase\u2010\u03b1 (G6Pase\u2010\u03b1), which facilitates microsomal G6P uptake by G6PT, fails to stimulate G6P uptake in P\n                    <jats:sub>i<\/jats:sub>\n                    \u2010loaded G6PT\u2010proteoliposomes, suggesting that the G6Pase\u2010\u03b1\u2010mediated stimulation is caused by decreasing G6P and increasing P\n                    <jats:sub>i<\/jats:sub>\n                    concentrations in microsomes. Taken together, our results suggest that G6PT has a dual role as a G6P and a P\n                    <jats:sub>i<\/jats:sub>\n                    transporter and that GSD\u2010Ib and GSD\u2010Ic are deficient in the same\n                    <jats:italic>G6PT<\/jats:italic>\n                    gene.\u2014Chen, S.\u2010Y., Pan, C.\u2010J., Nandigama, K., Mansfield, B., Ambudkar, S., Chou, J. The glucose\u20106\u2010phosphate transporter is a phosphate\u2010linked antiporter deficient in glycogen storage disease type Ib and Ic.\n                    <jats:italic>FASEB J.<\/jats:italic>\n                    22, 2206\u20132213 (2008)\n                  <\/jats:p>","DOI":"10.1096\/fj.07-104851","type":"journal-article","created":{"date-parts":[[2008,3,12]],"date-time":"2008-03-12T20:59:30Z","timestamp":1205355570000},"page":"2206-2213","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":63,"title":["The glucose\u20106\u2010phosphate transporter is a phosphate\u2010linked antiporter deficient in glycogen storage disease type Ib and Ic"],"prefix":"10.1096","volume":"22","author":[{"given":"Shih-Yin","family":"Chen","sequence":"first","affiliation":[{"name":"Section on Cellular Differentiation Heritable Disorders Branch National Institute of Child Health and Human Development National Institutes of Health Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chi-Jiunn","family":"Pan","sequence":"additional","affiliation":[{"name":"Section on Cellular Differentiation Heritable Disorders Branch National Institute of Child Health and Human Development National Institutes of Health Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Krishnamachary","family":"Nandigama","sequence":"additional","affiliation":[{"name":"Laboratory of Cell Biology Center for Cancer Research National Cancer Institute National Institutes of Health Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian C.","family":"Mansfield","sequence":"additional","affiliation":[{"name":"Section on Cellular Differentiation Heritable Disorders Branch National Institute of Child Health and Human Development National Institutes of Health Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suresh V.","family":"Ambudkar","sequence":"additional","affiliation":[{"name":"Laboratory of Cell Biology Center for Cancer Research National Cancer Institute National Institutes of Health Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janice Y.","family":"Chou","sequence":"additional","affiliation":[{"name":"Section on Cellular Differentiation Heritable Disorders Branch National Institute of Child Health and Human Development National Institutes of Health Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2008,3,12]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.2174\/1566524024605798"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)45462-8"},{"key":"e_1_2_4_4_1","doi-asserted-by":"crossref","first-page":"4901","DOI":"10.1016\/S0021-9258(17)33200-3","article-title":"Microsomal membrane permeability and the hepatic glucose-6- phosphatase system","volume":"251","author":"Arion W. 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