Literature DB >> 6477509

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.   

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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Year:  1984        PMID： 6477509      PMCID： PMC1144139          DOI： 10.1042/bj2220025

Source DB:  PubMed          Journal:  Biochem J        ISSN： 0264-6021            Impact factor:   3.857


  13 in total

1.  A RAPID PERMETHYLATION OF GLYCOLIPID, AND POLYSACCHARIDE CATALYZED BY METHYLSULFINYL CARBANION IN DIMETHYL SULFOXIDE.

Authors:  S HAKOMORI
Journal:  J Biochem       Date:  1964-02       Impact factor: 3.387

2.  Human liver alpha-L-fucosidases.

Authors:  D Robinson; R Thorpe
Journal:  Clin Chim Acta       Date:  1973-09-14       Impact factor: 3.786

3.  Disposition of fucose in brain.

Authors:  R K Margolis; R U Margolis
Journal:  J Neurochem       Date:  1972-04       Impact factor: 5.372

4.  Oligosaccharide storage in brains from patients with fucosidosis, GM1-gangliosidosis and GM2-gangliosidosis (Sandhoff's disease).

Authors:  G C Tsay; G Dawson
Journal:  J Neurochem       Date:  1976-09       Impact factor: 5.372

5.  Neuronal storage disease in English springer spaniels.

Authors:  J D Littlewood; M E Herrtage; A C Palmer
Journal:  Vet Rec       Date:  1983-01-22       Impact factor: 2.695

6.  Canine alpha-L-fucosidosis: a storage disease of Springer Spaniels.

Authors:  W R Kelly; A E Clague; R J Barns; M J Bate; B M MacKay
Journal:  Acta Neuropathol       Date:  1983       Impact factor: 17.088

7.  A suspected new canine storage disease.

Authors:  W J Hartley; P J Canfield; T M Donnelly
Journal:  Acta Neuropathol       Date:  1982       Impact factor: 17.088

8.  Electrophoretic forms of human liver alpha-L-fucosidase and their relationship to fucosidosis (mucopolysaccharidosis F).

Authors:  J A Alhadeff; A L Miller; D A Wenger; J S O'Brien
Journal:  Clin Chim Acta       Date:  1974-12-17       Impact factor: 3.786

9.  Urinary glycopeptides of fucosidosis.

Authors:  K Yamashita; Y Tachibana; S Takada; I Matsuda; S Arashima; A Kobata
Journal:  J Biol Chem       Date:  1979-06-10       Impact factor: 5.157

10.  Urinary oligosaccharides of fucosidosis. Evidence of the occurrence of X-antigenic determinant in serum-type sugar chains of glycoproteins.

Authors:  M Nishigaki; K Yamashita; I Matsuda; S Arashima; A Kobata
Journal:  J Biochem       Date:  1978-10       Impact factor: 3.387
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  8 in total

1.  GM1 gangliosidosis (type 1) in a cat.

Authors:  C G Barker; W F Blakemore; A Dell; A C Palmer; P R Tiller; B G Winchester
Journal:  Biochem J       Date:  1986-04-01       Impact factor: 3.857

2.  Lectin histochemistry of gangliosidosis. I. Neural tissue in four mammalian species.

Authors:  J Alroy; V Goyal; C D Warren
Journal:  Acta Neuropathol       Date:  1988       Impact factor: 17.088

3.  Canine alpha-L-fucosidase in relation to the enzymic defect and storage products in canine fucosidosis.

Authors:  C Barker; A Dell; M Rogers; J A Alhadeff; B Winchester
Journal:  Biochem J       Date:  1988-09-15       Impact factor: 3.857

4.  Human and canine fucosidosis: a comparative lectin histochemistry study.

Authors:  J Alroy; A A Ucci; C D Warren
Journal:  Acta Neuropathol       Date:  1985       Impact factor: 17.088

5.  Structural analysis of the major urinary oligosaccharides in feline alpha-mannosidosis.

Authors:  D Abraham; P Daniel; A Dell; J Oates; R Sidebotham; B Winchester
Journal:  Biochem J       Date:  1986-02-01       Impact factor: 3.857

6.  The molecular defect underlying canine fucosidosis.

Authors:  B J Skelly; D R Sargan; M E Herrtage; B G Winchester
Journal:  J Med Genet       Date:  1996-04       Impact factor: 6.318

7.  A mouse model for fucosidosis recapitulates storage pathology and neurological features of the milder form of the human disease.

Authors:  Heike Wolf; Markus Damme; Stijn Stroobants; Rudi D'Hooge; Hans Christian Beck; Irm Hermans-Borgmeyer; Renate Lüllmann-Rauch; Thomas Dierks; Torben Lübke
Journal:  Dis Model Mech       Date:  2016-08-04       Impact factor: 5.758

8.  Canine GM2-Gangliosidosis Sandhoff Disease Associated with a 3-Base Pair Deletion in the HEXB Gene.

Authors:  P Wang; P S Henthorn; E Galban; G Lin; T Takedai; M Casal
Journal:  J Vet Intern Med       Date:  2017-11-06       Impact factor: 3.333
  8 in total

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