Deficiency of alpha-mannosidase in Angus cattle. An inherited lysosomal storage disease

A disease of Angus cattle previously known as pseudolipidosis has been shown to be an inherited lysosomal storage disease, in which an oligosaccharide containing mannose and glucosamine is the storage substance. Diseased animals have a near-absolute deficiency of the lysosomal enzyme, alpha-mannosid...

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Bibliographic Details
Published in:Biochemical journal 1972-06, Vol.128 (1), p.69-78
Main Authors: Hocking, J D, Jolly, R D, Batt, R D
Format: Article
Language:English
Subjects:
Animals
Brain - enzymology
Cattle
Cattle Diseases - enzymology
Chick Embryo
Chromatography, Paper
Female
Glucosamine
Hexosaminidases
Hexoses
Lipid Metabolism, Inborn Errors - veterinary
Liver - enzymology
Lymph Nodes - enzymology
Lysosomes - metabolism
Male
Mannose
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Summary:A disease of Angus cattle previously known as pseudolipidosis has been shown to be an inherited lysosomal storage disease, in which an oligosaccharide containing mannose and glucosamine is the storage substance. Diseased animals have a near-absolute deficiency of the lysosomal enzyme, alpha-mannosidase, whereas heterozygotes have a partial deficiency of this enzyme. The condition is analogous to the human disease known as mannosidosis.
ISSN:0264-6021
0306-3283
1470-8728
DOI:10.1042/bj1280069