Cerebral and visceral organ gangliosides and related glycolipids in gm1-gangliosidosis type 1, type 2 and chronic type.
Biochemical analyses of the samples of GM1-gangliosidosis Type 1, Type 2, and Chronic type at autopsy showed that GM1 and asialo-GM1 are markedly increased in the whole cerebral tissues of patients with Type 1 and Type 2, but mainly in the basal ganglia including caudate nucleus and putamen in the Chronic Type as already reported by Kobayashi and Suzuki in 1981. On the other hand, the finding that the Type 1 visceral organs contained unusual glycolipids rather than gangliosides was contrasted to those of Type 2 which seemed less abnormal. The unusual glycolipids included particularly fucolipid I and II consisting of glucose:galactose:N-acetylglucosamine:fucose (1:2:1:1) and (1:3:2:2), respectively. The chemical structure of oligosaccharide moiety of fucolipid I may be identical with Lacto-N-fucopentaose II or Lacto-N-fucopentaose III. The fucolipid II may be proposed to be Lacto-N-difucooctanose.
D001923:Brain Chemistry; D002554:Cerebrosides / Q000032:analysis; D002675:Child, Preschool; D002855:Chromatography, Thin Layer / Q000379:methods; D005260:Female; D005677:G(M1) Ganglioside / Q000378:metabolism*; D005732:Gangliosides / Q000032:analysis* / Q000378:metabolism*; D005733:Gangliosidoses / Q000378:metabolism*; D006017:Glycolipids / Q000032:analysis*; D006801:Humans; D007668:Kidney / Q000032:analysis*; D008055:Lipids / Q000032:analysis; D008099:Liver / Q000032:analysis*; D013154:Spleen / Q000032:analysis*
D002554:Cerebrosides; D005732:Gangliosides; D006017:Glycolipids; D008055:Lipids; D005677:G(M1) Ganglioside