alfaglukozidáz

Alpha-glucosidase is an enzyme that plays a crucial role in the digestion of carbohydrates. It belongs to the glycoside hydrolase family and is responsible for breaking down complex carbohydrates, specifically oligosaccharides and disaccharides, into simpler monosaccharides, such as glucose. This process is essential for the absorption of these sugars into the bloodstream.

The primary location of alpha-glucosidase activity in mammals is the brush border of the small intestine. Here, it works in conjunction with other enzymes like sucrase and maltase to break down dietary sugars like sucrose, maltose, and isomaltose. These smaller sugars can then be readily absorbed by the intestinal cells.

Genetic deficiencies in alpha-glucosidase can lead to glycogen storage diseases. One notable example is Pompe disease, a rare genetic disorder caused by insufficient activity of lysosomal alpha-glucosidase (also known as acid alpha-glucosidase). This deficiency leads to the accumulation of glycogen in lysosomes, particularly in muscle cells, causing progressive muscle weakness and damage. Enzyme replacement therapy is available for certain forms of Pompe disease.

In summary, alpha-glucosidase is a vital enzyme for carbohydrate metabolism, facilitating the breakdown of complex sugars into absorbable glucose. Its malfunction can result in serious health conditions like glycogen storage diseases.