{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,9]],"date-time":"2026-05-09T02:03:22Z","timestamp":1778292202263,"version":"3.51.4"},"reference-count":28,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2004,10,7]],"date-time":"2004-10-07T00:00:00Z","timestamp":1097107200000},"content-version":"vor","delay-in-days":6946,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[1985,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We describe neurochemical abnormalities found in the brains of 2 patients with autopsy\u2010confirmed Hallervorden\u2010Spatz (HS) disease. In 1 patient, contents of cystine and of glutathione\u2010cysteine mixed disulfide in the globus pallidus were markedly elevated above values for appropriate control subjects. Activity of cysteine dioxygenase, which converts cysteine to cysteine sulfinic acid, was reduced in the globus pallidus, but normal in the frontal cortex and putamen of both patients. \u03b3\u2010Aminobutyric acid content was markedly decreased in the globus pallidus and substantia nigra of both patients. These results suggest that cysteine accumulates locally in the globus pallidus in Hallervorden\u2010Spatz disease as a result of an enzymatic block in the metabolic pathway from cysteine to taurine. Accumulated cysteine may chelate iron, accounting for the local increase in iron content in Hallervorden\u2010Spatz disease. The combined excess of cysteine and ferrous iron may generate free radicals that damage neuronal membranes to cause the typical morphological changes observed in this disorder.<\/jats:p>","DOI":"10.1002\/ana.410180411","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T17:26:24Z","timestamp":1104600384000},"page":"482-489","source":"Crossref","is-referenced-by-count":147,"title":["Hallervorden\u2010Spatz disease: Cysteine accumulation and cysteine dioxygenase deficiency in the globus pallidus"],"prefix":"10.1002","volume":"18","author":[{"given":"Thomas L","family":"Perry","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Margaret G.","family":"Norman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Voon Wee","family":"Yong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sharon","family":"Whiting","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John U.","family":"Crichton","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shirley","family":"Hansen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephen J.","family":"Kish","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,10,7]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/102.4.727"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(73)90114-7"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1974.00490310072012"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1979.tb00842.x"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.1975.tb11895.x"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(73)90162-7"},{"key":"e_1_2_1_8_2","first-page":"106","article-title":"Nonspecific accumulation of metals in the globus pallidus in Hallervorden\u2010Spatz disease","volume":"104","author":"Goldberg W","year":"1979","journal-title":"Trans Am Neurol Assoc"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF02878455"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.35.2.227"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.31.11.1453"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-196707000-00001"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)52451-6"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/0020-711X(79)90033-8"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.1971.tb11979.x"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.1981.tb01608.x"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.1975.tb07680.x"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.1982.tb08697.x"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.31.3.237"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.1016\/0021-9673(68)85074-5"},{"key":"e_1_2_1_21_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.1984.tb12834.x"},{"key":"e_1_2_1_22_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00390234"},{"key":"e_1_2_1_23_2","first-page":"1844","volume-title":"The Metabolic Basis of Inherited Disease. 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