Structural and Mechanistic Studies of alpha-galactosidase A and Pharmacological Chaperones
Human α-galactosidase (α-GAL; EC 3.2.1.22) is a lysosomal enzyme that hydrolyzes of terminal alpha-linked galactosyl residue of glycosphingolipids. Deficiencies in α-GAL leads to Fabry disease, which is characterized by the build-up of globotriaosylceramide and other neutral substrates in cells, ult...
Main Author: | |
---|---|
Format: | Others |
Published: |
ScholarWorks@UMass Amherst
2010
|
Subjects: | |
Online Access: | https://scholarworks.umass.edu/open_access_dissertations/202 https://scholarworks.umass.edu/cgi/viewcontent.cgi?article=1202&context=open_access_dissertations |