Alpha-galactosidase
(Redirected from Replagal)
Alpha-galactosidase is an enzyme that hydrolyzes alpha-galactosides, including melibiose, raffinose, and stachyose. It is encoded by the GLA gene in humans. Alpha-galactosidase is a glycoside hydrolase enzyme that breaks down complex sugars derived from plants.
Etymology[edit | edit source]
The term "alpha-galactosidase" is derived from the Greek words "alpha," meaning "first," "galactos," meaning "milk," and "sidase," meaning "enzyme." It refers to the enzyme's ability to break down alpha-galactosides, a type of sugar found in many plants and some milk products.
Function[edit | edit source]
Alpha-galactosidase is a lysosomal enzyme that breaks down glycosphingolipids, specifically globotriaosylceramide, into glucose and ceramide. It is active in the lysosomes, where it breaks down glycosphingolipids, which are complex molecules that are part of the cell membrane and also have functions in cell-cell interactions.
Clinical significance[edit | edit source]
Deficiency of alpha-galactosidase causes Fabry disease, a rare genetic disorder that can damage major organs and shorten lifespan. Symptoms of Fabry disease can range from mild to severe and may appear anytime from childhood to adulthood.
See also[edit | edit source]
References[edit | edit source]
Alpha-galactosidase Resources | |
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