{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T13:45:37Z","timestamp":1790516737317,"version":"4.1.0"},"reference-count":23,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2004,11,4]],"date-time":"2004-11-04T00:00:00Z","timestamp":1099526400000},"content-version":"vor","delay-in-days":2256,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[1998,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Previous studies of patients with spinocerebellar atrophy type 1 (SCA\u20101) and Friedreich's ataxia (FA) have suggested the occurrence of membrane distrubances in both disorders. We measured concentrations of phosphatidylcholine (PC), diacyl and plasmalogen phosphatidylethanolamine (PE), and phosphatidylserine (PS), along with their fatty acid profiles, in the brains of eight patients with Friedreich's ataxia (FA) and nine patients with dominantly inherited spinocerebellar atrophy type 1 (SCA\u20101). Compared with the controls, levels of all phospholipid types (PE, PS, and PC) were reduced in the cerebellar but not occipital cortex of SCA\u20101 patients. In contrast, in the FA group, levels of PS and PE, but not PC, were reduced in both cerebellar and occipital cortices. The fatty acid composition of individual brain phospholipids was altered in both FA and SCA\u20101 patients, most markedly in the plasmalogen PE and PS classes of cerebellar phospholipids. Given the neuropathologic characteristics of each disorder, it is likely that altered fatty acid composition and phospholipid levels in SCA\u20101 cerebellar cortex occur as a consequence of pronounced cerebellar degeneration. In contrast, reduced phospholipid levels in FA cerebellar and occipital cortex, areas characterized by, at most, minimal neuronal loss in FA, may represent a widespread alteration in cellular phospholipid metabolism occurring in response to the specific gene defect in the disorder.<\/jats:p>","DOI":"10.1002\/mds.870130510","type":"journal-article","created":{"date-parts":[[2004,12,31]],"date-time":"2004-12-31T09:49:30Z","timestamp":1104486570000},"page":"813-819","source":"Crossref","is-referenced-by-count":21,"title":["Brain phospholipids and fatty acids in Friedreich's ataxia and spinocerebellar atrophy type\u20101"],"prefix":"10.1002","volume":"13","author":[{"given":"Klaus","family":"Eder","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen J.","family":"Kish","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manfred","family":"Kirchgessner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brain M.","family":"Ross","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2004,11,4]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng0793-221"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00318569"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.271.5254.1423"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100035113"},{"key":"e_1_2_1_6_2","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1017\/S0317167100024951","article-title":"Plasma lipids and lipoproteins in Friedreich's ataxia and familial spastic ataxia: evidence for an abnormal composition of high density lipoproteins","volume":"5","author":"Huang YS","year":"1978","journal-title":"Can J Neurol Sci"},{"key":"e_1_2_1_7_2","first-page":"382","article-title":"Plasma lipoprotein concentration and erythocyte membrane lipids in patients with Friedreich's ataxia","volume":"2","author":"Filla A","year":"1980","journal-title":"Acta Neurol"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100119778"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100023015"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100119791"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/0304-3940(90)90040-G"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/0304-3940(93)90291-R"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/0304-4157(84)90005-4"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.89.5.1671"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.1993.tb07436.x"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(78)90046-5"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/0021-9673(92)87054-C"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1093\/jn\/124.10.1917"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9673(00)81946-9"},{"key":"e_1_2_1_20_2","first-page":"101","article-title":"Studies of phospholipid composition in Alzheimer's disease brain","volume":"2","author":"Kienzl E","year":"1993","journal-title":"Neurodegeneration"},{"key":"e_1_2_1_21_2","doi-asserted-by":"crossref","first-page":"2227","DOI":"10.1093\/ajcn\/32.11.2227","article-title":"Changes in content and composition of brain phospholipids in malnourished children","volume":"32","author":"Yusuf HK","year":"1979","journal-title":"Am J Clin Nutr"},{"key":"e_1_2_1_22_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.1995.64052213.x"},{"key":"e_1_2_1_23_2","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1007\/978-1-4615-5405-9_6","volume-title":"Neurochemistry. 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