Aspartylglucosylamine deaspartylase

Aspartylglucosylamine Deaspartylase is an enzyme that plays a critical role in the metabolism of certain amino acids in the human body. This enzyme is involved in the breakdown of aspartylglucosylamine, a compound that is formed during the metabolism of proteins.

Function

Aspartylglucosylamine Deaspartylase is responsible for the hydrolysis of aspartylglucosylamine, a process that results in the release of aspartic acid and glucosamine. This reaction is a crucial step in the lysosomal degradation of glycoproteins.

Clinical Significance

Deficiency in Aspartylglucosylamine Deaspartylase can lead to a rare genetic disorder known as Aspartylglucosaminuria. This condition is characterized by the accumulation of aspartylglucosylamine in body fluids and tissues, leading to progressive intellectual disability, skeletal abnormalities, and other health problems.

Structure and Mechanism

The exact structure and mechanism of Aspartylglucosylamine Deaspartylase are not fully understood. However, it is believed that the enzyme functions as a hydrolase, using water to break the bond between aspartic acid and glucosamine in aspartylglucosylamine.

See Also

• Aspartylglucosaminuria
• Enzyme
• Metabolism
• Amino Acid
• Protein
• Glycoprotein