Lysosomal storage disease mannosidosis

Golgi a-l)-mannosidase II lysosomal mannosidase glycoprotein A-linked oligosaccharide processing [toxic, neurotoxic effects mimic hereditary lysosomal storage disease mannosidosis] (3-D-Glucuronidase cx-1-iduronidase Glucosidase... 

This and similar methods have been used to identify a number of oligosaccharides from natural sources [2,334]. One example of some clinical interest is the structural analysis of a-D-mannopyranoside-(l 3)-/3-D-mannopyranoside-(l ->4)-a-acetamido-a-deoxy-D-glucose in the urine from patients with a lysosomal storage disease, mannosidosis [335]. 

Levels of lysosomal j8-D-2-acetamido-2-deoxyhexosidase have been determined in normal human hair roots and in hair roots obtained from a patient with mannosidosis. The significance of the results was discussed in relation to the levels of other glycoside hydrolases present, and to the detection of lysosomal storage disease. The f3-D-2-acetamido-2-deoxyhexosidase was found to be the most active glycoside hydrolase in normal hair roots. 

Before swainsonine could be used clinically, the possible adverse effects of this agent had to be evaluated. As previously discussed, the systemic administration of high doses of swainsonine to sheep has been reported to induce a neural lysosomal mannoside storage disease.However, no evidence of an overt toxic reaction was observed when swainsonine was administered orally to rodents so it was considered that the mannosidosis induced by swainsonine could be a species- or tissue-specific... 

---