{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,20]],"date-time":"2025-09-20T21:06:21Z","timestamp":1758402381290},"reference-count":20,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T00:00:00Z","timestamp":1152576000000},"content-version":"vor","delay-in-days":6888,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1987,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The oxidized form of glutathione transport was studied in human erythrocytes in pyrimidine 5\u2032\u2010nucleotidase (P5N) deficiency, a disorder in which the amounts of CTP and UTP in the erythrocytes are elevated. The inhibition of ATP\u2010requiring oxidized glutathione (GSSG) transport by CTP and UTP is believed to play a role in elevating the levels of the reduced form of glutathione (GSH) in the erythrocytes of patients with P5N deficiency. The current investigation was undertaken to determine if GSSG transport actually decreases in the erythrocytes of such patients. Erythrocytes from a 17\u2010year\u2010old patient and a 13\u2010year\u2010old patient with P5N deficiency hemolytic anemia and from ten normal subjects were used as materials for the experiment. Erythrocytes, which had been previously incubated with [<jats:sup>3<\/jats:sup>H]glycine, were incubated at 37\u00b0C, and the rate of [<jats:sup>3<\/jats:sup>H]GSSG transported by the cells was estimated. The velocity of GSSG transport out of the erythrocytes was quite low in the patients, 3.17\u20133.65 nmol GSSG\/ml erythrocytes\/hr at 37\u00b0C in one case, and 3.30 nmol GSSG\/ml erythrocytes\/hr in the other case, vs that in the normal controls (6.00 \u00b1 0.80 nmol GSSG\/ml erythrocytes\/hr; mean \u00b1 SD). The activity of \u03b3\u2010glutamylcysteine synthetase and glutathione synthetase did not decrease in the patients. Decreased transport activity of GSSG in addition to a normal synthesis rate for GSH may explain the increased concentration of erythrocyte GSH in P5N deficiency.<\/jats:p>","DOI":"10.1002\/ajh.2830260105","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T11:05:14Z","timestamp":1172055914000},"page":"37-45","source":"Crossref","is-referenced-by-count":11,"title":["Erythrocyte\u2010oxidized glutathione transport in pyrimidine 5\u2032\u2010nucleotidase deficiency"],"prefix":"10.1002","volume":"26","author":[{"given":"Takahito","family":"Kondo","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshinori","family":"Ohtsuka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michiro","family":"Shimada","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshikazu","family":"Kawakami","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasuhiko","family":"Hiyoshi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshiro","family":"Tsuji","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hisaichi","family":"Fujii","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shiro","family":"Miwa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,7,11]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI107826"},{"key":"e_1_2_1_3_2","first-page":"563","article-title":"A 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