Garman lab: Interconversion of lysosomal enzyme specificities
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Lysosomal storage disease
Immune Response
Individuals suffering from Fabry disease cannot produce the Alpha-Gal protein that is necessary for breaking down Galactose. The usual treatment for this is giving the patient doses of the protein, but this poses a problem. Since the body does not produce the protein, an immune response ranging from severe anaphylaxis to mild discomfort can occur when the patient is given the protein. The body does however produce Alpha NAGAL, a protein with a similar active site and function as Alpha Gal. Altering the active site of Alpha NAGAL to match that of Alpha Gal allows doctors to administer a protein that serves the function of Alpha Gal but has the antigenicity of Alpha NAGAL, which means the body will recognize the protein and not elicit an immune response.
Enzymatic activity
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Structures shown on this page
3H54: alpha-NAGAL with GalNAc
3HG5: alpha-GAL with Gal
3LX9: alpha-GAL(SA) in complex with GalNAc
3LXA: alpha-GAL(SA) in complex with Galactose
3LXC: alpha-GAL(SA) in presence of Glycerol
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