Title
Caprine oligosaccharide storage disease. Accumulation of β-mannosyl (1→4) β-N-acetylglucosaminyl (1→4) β-N-acetylglucosamine in brain
Document Type
Article
Publication Date
12-1-1981
Abstract
In 1975 a new caprine neurovisceral storage disease was identified in related Nubian goats in Michigan. The affected kids of both sexes showed profound neurological deficits at birth, lack of myelination in cerebral hemispheres and cerebellum, axonal lesions, and cytoplasmic vacuolation. A similar genetic syndrome arose independently in a population of Anglo-Nubian goats in New South Wales. Preliminary chemical characterization of an accumulated metabolite was performed. An extract of 1 g of brain from an affected goat was found to contain 2.2 μmol of the trisaccharide Man (β1→4)GlcNAc(β1→4)GlcNAc (β-mannosylchitobiose). The accumulation of this substance suggests the possibility of a genetic defect in β-mannosidase in the catabolic pathway for N-linked complex glycopeptides and would be the first indication of a β-mannosidosis.
Publication Source (Journal or Book title)
Journal of Biological Chemistry
First Page
5181
Last Page
5184
Recommended Citation
Jones, M., & Laine, R. (1981). Caprine oligosaccharide storage disease. Accumulation of β-mannosyl (1→4) β-N-acetylglucosaminyl (1→4) β-N-acetylglucosamine in brain. Journal of Biological Chemistry, 256 (10), 5181-5184. Retrieved from https://digitalcommons.lsu.edu/biosci_pubs/2231