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Structural comparison of differently glycosylated forms of acid-beta-glucosidase, the defective enzyme in Gaucher disease.

Abstract:

Gaucher disease is caused by mutations in the gene encoding acid-beta-glucosidase. A recombinant form of this enzyme, Cerezyme, is used to treat Gaucher disease patients by ;enzyme-replacement therapy'. Crystals of Cerezyme after its partial deglycosylation were obtained earlier and the structure was solved to 2.0 A resolution [Dvir et al. (2003), EMBO Rep. 4, 704-709]. The crystal structure of unmodified Cerezyme is now reported, in which a substantial number of sugar residues bound to three...

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Publication status:
Published

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Publisher copy:
10.1107/S0907444906038303

Authors


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Institution:
University of Oxford
Department:
Oxford, MSD, Biochemistry
Role:
Author
Journal:
Acta crystallographica. Section D, Biological crystallography
Volume:
62
Issue:
Pt 12
Pages:
1458-1465
Publication date:
2006-12-05
DOI:
EISSN:
1399-0047
ISSN:
0907-4449
URN:
uuid:772303b0-f895-4ca6-9744-9bff6baa132e
Source identifiers:
99973
Local pid:
pubs:99973

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