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

Biochem J. 1972 Jun;128(1):69-78. doi: 10.1042/bj1280069.

Abstract

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.

MeSH terms

  • 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

Substances

  • Hexoses
  • Hexosaminidases
  • Glucosamine
  • Mannose