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. 1984 Aug 15;222(1):25–33. doi: 10.1042/bj2220025

The enzymic defect and storage products in canine fucosidosis.

D Abraham, W F Blakemore, A Dell, M E Herrtage, J Jones, J T Littlewood, J Oates, A C Palmer, R Sidebotham, B Winchester
PMCID: PMC1144139  PMID: 6477509

Abstract

A marked deficiency of alpha-L-fucosidase and the accumulation of fucose-containing glycoasparagines were found in the brains of two English Springer spaniels suffering from a progressive nervous disorder. Both forms of alpha-L-fucosidase in normal brain, which are separable by ion-exchange chromatography, are absent from the affected animals. The storage products were characterized by t.l.c., gel filtration, g.l.c. and fast-atom-bombardment mass spectrometry. The postulated structures of the main components are: (formula; see text) The enzymic defect and nature of storage products justify designation of this disorder as canine fucosidosis.

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Selected References

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