Hepatic storage of oligosaccharides and glycolipids in a cat affected with GM1 gangliosidosis.
1. Glycopeptides and glycolipids were isolated from normal cat liver and liver from a cat affected with GM1 gangliosidosis. 2. Bio-Gel P-6 chromatography of the crude glycopeptide fractions demonstrated three major peaks of hexose-containing compounds that were greatly increased in the mutant liver sample; these peaks contained oligosaccharides that comprised over 2% of the liver wet weight. 3. Two of the major pathological oligosaccharides, GP5 and GP6, were purified by chromatography on charcoal/Celite and Sephadex G-25. Oligosaccharides GP5 and GP6 had apparent mol.wts. of 1800 +/- 200 and 1350+/-200 respectively, and contained galactose, mannose and N-acetylglucosamine in molar proportions of 2.0:3.1:4.1 (GP5) and 1.0:2.2:2.7 (GP6). Periodate oxidation studies demonstrated the presence of galactose in a non-reducing terminal position. 4. The neutral glycolipid fraction from the mutant cat liver has a 1.3-fold increase in hexose content accompanied by an increased concentration of asialo-(ganglioside GM1). 5. There was a 2-fold increase of gangliosides in the mutant cat liver compared with normal cats. Ganglioside GM1 and a compound tentatively identified as N-glycolloyl-(ganglioside GM1) were the major glycolipids accumulated.
D000818:Animals; D002241:Carbohydrates / Q000032:analysis; D002415:Cats; D002850:Chromatography, Gel; D002855:Chromatography, Thin Layer; D005733:Gangliosidoses / Q000378:metabolism*; D006017:Glycolipids / Q000378:metabolism*; D006801:Humans; D008099:Liver / Q000378:metabolism*; D009844:Oligosaccharides / Q000378:metabolism*; D010504:Periodic Acid
D002241:Carbohydrates; D006017:Glycolipids; D009844:Oligosaccharides; D010504:Periodic Acid