The enzymic defect and storage products in canine fucosidosis
Keyword(s):
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.
1973 ◽
Vol 30
(02)
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pp. 414-424
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1984 ◽
Vol 51
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pp. 016-021
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1990 ◽
Vol 36
(11)
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pp. 1906-1910
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2021 ◽
Vol 13
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pp. 107-112
2004 ◽
Vol 19
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pp. 1309
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