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AAT Bioquest

What are the functions of alpha galactosidase?

Posted December 7, 2023


Answer

Alpha galactosidase is an enzyme which catalyzes the hydrolysis of a-galactosidase in the lysosomes. They function to prevent gas and bloating which can occur after consuming these foods through breaking down the carbohydrates associated with it. Abnormalities in human alpha galactosidase can result in a medical condition known as Fabry disease. Fabry disease is a type of lysosomal storage disorder, and occurs when there is an absence or deficiency of a-galachoglyated substrates in the body’s tissues (due to mutations in the GLA gene). 

Additional resources

Structure–function relationships in α-galactosidase A

Enzymes

Alpha-galactosidase A Inhibitors (IC50, Ki)