Beta-galactosidase

Beta-galactosidase is an enzyme that catalyzes the hydrolysis of beta-galactosides into monosaccharides. It is a crucial enzyme in the human body, playing a significant role in the metabolism of lactose, a sugar found in milk and other dairy products.

Function

Beta-galactosidase is responsible for breaking down complex sugars into simpler sugars that the body can use for energy. It does this by cleaving the glycosidic bond in beta-galactosides, a type of sugar molecule. This process is essential for the digestion of lactose in the human body.

Structure

The enzyme is a tetramer, meaning it is composed of four subunits. Each subunit contains a catalytic domain and a domain for binding to the substrate. The catalytic domain is responsible for the enzymatic activity of beta-galactosidase, while the substrate-binding domain ensures the enzyme binds to the correct molecule.

Clinical significance

Deficiency in beta-galactosidase can lead to a number of medical conditions. For example, it can cause galactosemia, a disorder that affects the body's ability to process galactose, a sugar found in milk and other dairy products. This can lead to a buildup of galactose in the blood, which can cause damage to the liver, kidneys, and brain.

Another condition caused by beta-galactosidase deficiency is GM1 gangliosidosis, a rare inherited disorder that progressively destroys nerve cells in the brain and spinal cord.

See also

• Lactase
• Galactosemia
• GM1 gangliosidosis

References

Beta-galactosidase Resources
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