Synthesis and testing of isofagomine and derivatives as inhibitors of human glucocerebrosidase

Gaucher disease is a lysosomal storage disorder in which the activity of the enzyme glucocerebrosidase (GCase) is deficient. As a result, the substrate of GCase, glucosylceramide, accumulates in lysosomes of Gaucher patients, causing enlargement of the spleen and liver, bone deformity and neurode...

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Bibliographic Details
Main Author: Hill, Tara
Language:English
Published: University of British Columbia 2009
Online Access:http://hdl.handle.net/2429/7568

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