Recombinant Human AGA protein (His tag)

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

The AGA protein also known as Aspartylglucosaminidase has an important role in catalyzing the hydrolysis of glycosylasparagine to glucosamine and aspartic acid. This activity occurs predominantly in the lysosomes. The AGA gene encodes this enzyme which has a molecular mass of approximately 35 kDa. It is expressed in a variety of tissues including the liver and kidney where it contributes significantly to protein degradation.

Biological function summary

Enzymes like AGA help in glycoprotein catabolism which is an essential process for cellular maintenance and function. AGA functions as a homodimer and is an integral part of the lysosomal enzyme complex. This complex ensures proper degradation of N-linked oligosaccharides from glycoproteins following cellular internalization. Disruption in AGA's function results in accumulation of undigested glycoproteins which may affect normal cellular processes.

Pathways

AGA's activity ties into the lysosomal degradation pathway significantly influencing the catabolic reduction of glycoproteins. It is closely related to other lysosomal enzymes such as cathepsins. AGA’s regulation is linked to cellular recycling and energy balance pathways transitioning the breakdown products of glycoproteins into reusable components for other metabolic processes.

Associated diseases and disorders

AGA is closely related to the condition known as Aspartylglucosaminuria. This rare inherited metabolic disorder results in the accumulation of glycoasparagines due to AGA deficiency leading to various symptoms including intellectual disability and skeletal abnormalities. In this disorder AGA malfunctions can also influence the activity of other lysosomal enzymes exacerbating the cellular effects of the accumulated substrates.