The enzymic defect and storage products in canine fucosidosis.
A marked deficiency of alpha-L-fucosidase and the accumulation of fucose-containing glycoasparagines were found in the brains of two English Springer spaniels suffering from a progressive nervous disorder. Both forms of alpha-L-fucosidase in normal brain, which are separable by ion-exchange chromatography, are absent from the affected animals. The storage products were characterized by t.l.c., gel filtration, g.l.c. and fast-atom-bombardment mass spectrometry. The postulated structures of the main components are: (formula; see text) The enzymic defect and nature of storage products justify designation of this disorder as canine fucosidosis.
D000818:Animals; D001921:Brain / Q000378:metabolism*; D050260:Carbohydrate Metabolism; D002239:Carbohydrate Metabolism, Inborn Errors / Q000378:metabolism / Q000662:veterinary*; D055598:Chemical Phenomena; D002621:Chemistry; D002848:Chromatography, DEAE-Cellulose; D002850:Chromatography, Gel; D002855:Chromatography, Thin Layer; D004195:Disease Models, Animal; D004283:Dog Diseases / Q000378:metabolism*; D004285:Dogs; D005643:Fucose / Q000378:metabolism*; D006026:Glycoside Hydrolases / Q000378:metabolism; D006863:Hydrogen-Ion Concentration; D008297:Male; D013058:Mass Spectrometry; D009844:Oligosaccharides / Q000378:metabolism; D005644:alpha-L-Fucosidase / Q000172:deficiency*
D009844:Oligosaccharides; D005643:Fucose; D006026:Glycoside Hydrolases; D005644:alpha-L-Fucosidase