{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T23:01:16Z","timestamp":1777417276017,"version":"3.51.4"},"reference-count":39,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2006,10,4]],"date-time":"2006-10-04T00:00:00Z","timestamp":1159920000000},"content-version":"vor","delay-in-days":10990,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Neurochemistry"],"published-print":{"date-parts":[[1976,9]]},"abstract":"<jats:p><jats:bold>Abstract\u2014<\/jats:bold> Analysis of whole autopsy brain from a patient with fucosidosis (\u03b1\u2010fucosidase deficiency) revealed minor storage of H\u2010antigen glycolipid [Fuc (\u03b1, 1\u21922) Gal\u2010GlcNAc\u2010Gal\u2010Glc\u2010Ceramide] and a slightly abnormal ganglioside composition in the form of a two\u2010fold elevation of G<jats:sub>M1<\/jats:sub> and the presence of a fucose\u2010containing glycolipid (a minor component) which co\u2010migrated with G<jats:sub>D1a<\/jats:sub>. The major storage materials in fucosidosis brain were an oligosaccharide (Fuc\u2010Gal\u2010GlcNAc\u2010Man[Fuc\u2010Gal\u2010GlcNAc\u2010Man]\u2010ManGlcNAc) and a disaccharide [Fuc(\u03b1, 1\u21926)\u2010GlcNAc] in the approximate ratio of 5:1. Lesser amounts of a related oligosaccharide (Gal\u2010GlcNAc\u2010Man[Gal\u2010GlcNAc\u2010Man]\u2010Man\u2010GlcNAc) were isolated from the brain of patients with G<jats:sub>M1<\/jats:sub>\u2010gangliosidosis (Types I and II) where the major storage material is known to be G<jats:sub>M1<\/jats:sub>\u2010ganglioside (Gal (\u03b2, 1\u21923)GalNAc(<jats:italic>\u03b2<\/jats:italic>, 1\u21924) [NeuNAcf(\u03b1, 2\u21923) Gal(\u03b2, 1\u21924)Glc\u2010Ceramide). Similarly, a related oligosaccharide (GlcNAc\u2010Man [GlcNAc\u2010Man]\u2010Man\u2010GlcNAc) was isolated from the brain of a patient with a total deficiency of <jats:italic>N<\/jats:italic>\u2010acetyl\u2010<jats:italic>\u03b2<\/jats:italic>\u2010<jats:sc>d<\/jats:sc>\u2010hexosaminidase (Sandhoff variant of G<jats:sub>M2<\/jats:sub>\u2010gangliosidosis) where the major storage products are known to be G<jats:sub>M2<\/jats:sub>\u2010ganglioside (GalNAc (<jats:italic>\u03b2<\/jats:italic> 1\u21924) [NeuNAc (\u03b1, 2\u21923)Gal(<jats:italic>\u03b2<\/jats:italic>, 1\u21924)Glc\u2010Ceramine) and its asialo derivative. These studies indicate that glycoproteins containing at least 2 mol of <jats:sc>l<\/jats:sc>\u2010fucose per oligosaccharide unit are normally catabolized in human brain. Further, it appears that such glycoproteins are initially catabolized by an endo\u2010<jats:italic>N<\/jats:italic>\u2010acetylglucosaminidase to release an oligosaccharide which is then degraded by the sequential action of exo\u2010glycosidases.<\/jats:p>","DOI":"10.1111\/j.1471-4159.1976.tb10401.x","type":"journal-article","created":{"date-parts":[[2006,10,5]],"date-time":"2006-10-05T06:25:18Z","timestamp":1160029518000},"page":"733-740","source":"Crossref","is-referenced-by-count":51,"title":["OLIGOSACCHARIDE STORAGE IN BRAINS FROM PATIENTS WITH FUCOSIDOSIS, G<sub>M1<\/sub>\u2010GANGLIOSIDOSIS AND G<sub>M2<\/sub>\u2010GANGLIOSIDOSIS (SANDHOFF'S DISEASE)<sup>1<\/sup>"],"prefix":"10.1111","volume":"27","author":[{"given":"Grace Chen","family":"Tsay","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Glyn","family":"Dawson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,10,4]]},"reference":[{"key":"e_1_2_1_2_1","first-page":"48","volume":"91","author":"Adachi M.","year":"1971","journal-title":"Archs Path."},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)42870-6"},{"key":"e_1_2_1_4_1","doi-asserted-by":"crossref","first-page":"4786","DOI":"10.1016\/S0021-9258(19)43734-4","volume":"248","author":"Banerjee S. 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