Aminoacylase

Aminoacylase is a type of enzyme that plays a crucial role in the metabolism of amino acids. It is specifically involved in the breakdown of aminoacyl compounds, which are produced during protein digestion. Aminoacylase is found in many organisms, including humans, and its activity is essential for normal growth and development.

Function

Aminoacylase catalyzes the hydrolysis of N-acetyl amino acids to produce amino acids and acetate. This reaction is important in the metabolism of dietary proteins, as it allows the body to utilize the amino acids obtained from food. In addition to its role in protein digestion, aminoacylase is also involved in the synthesis of certain biomolecules, such as neurotransmitters and hormones.

Structure

The structure of aminoacylase is complex, consisting of multiple subunits that work together to catalyze the hydrolysis reaction. Each subunit contains an active site, where the substrate (the N-acetyl amino acid) binds and the reaction takes place. The structure of the enzyme is highly conserved across different species, indicating its importance in biological processes.

Clinical significance

Mutations in the gene encoding aminoacylase can lead to a rare metabolic disorder known as Aminoacylase deficiency. This condition is characterized by an inability to break down certain amino acids, leading to their accumulation in the body. Symptoms can include neurological abnormalities, developmental delay, and other health problems. Diagnosis is typically made through biochemical testing, and treatment is aimed at managing symptoms and preventing complications.

See also

• Enzyme
• Metabolism
• Amino acid
• Aminoacylase deficiency

References

Aminoacylase Resources
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