Biochemical studies on a case of feline mannosidosis.
Evidence is presented for the biochemical diagnosis of the first case of feline mannosidosis. A marked deficiency of acidic alpha-D-mannosidase in the brain, kidney and liver and excessive excretion of mannose-rich oligosaccharides in the urine were found in a kitten suffering from a nervous disorder. Residual acidic alpha-D-mannosidase, ranging from 2 to 5.5% of the normal activity, was observed in the tissues of the affected kitten. It has similar kinetic and physicochemical properties to the normal activity. The amount of mannose in the urine of the affected kitten was 19-fold greater than in a comparable control, and the molar ratio of mannose to N-acetylglucosamine was approx. 6 : 1. High concentrations of neutral oligosaccharides were detected in the urine. The predominant oligosaccharide appeared to be a hexasaccharide. The biochemical features of bovine, feline and human mannosidosis are compared, and it is concluded that feline mannosidosis may be a useful animal model for studying the human disease.
D000818:Animals; D002239:Carbohydrate Metabolism, Inborn Errors / Q000378:metabolism / Q000662:veterinary*; D002371:Cat Diseases / Q000378:metabolism*; D002415:Cats; D002848:Chromatography, DEAE-Cellulose; D005260:Female; D006863:Hydrogen-Ion Concentration; D008247:Lysosomes / Q000201:enzymology; D008358:Mannose / Q000378:metabolism*; D008361:Mannosidases / Q000172:deficiency / Q000378:metabolism; D009844:Oligosaccharides / Q000652:urine
D009844:Oligosaccharides; D008361:Mannosidases; D008358:Mannose