alpha-Mannosidase Assay Kit

補足情報

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Alpha-Mannosidase also known as α-mannosidase or MAN2A1 is an enzyme that plays an important role in the processing of glycoproteins. This enzyme catalyzes the hydrolysis of alpha-mannosidic linkages in oligosaccharides. It has a molecular mass of approximately 125 kDa. Alpha-Mannosidase is expressed in various tissues throughout the human body including the liver kidney and brain. Its activity is especially high in the lysosomal compartment of cells where it participates in the breakdown of glycoproteins.

Biological function summary

The enzyme participates in the modification of N-linked glycoproteins during their processing in the endoplasmic reticulum and Golgi apparatus. It acts as part of a complex process that ensures proper folding and function of glycoproteins. Efficient activity of alpha-Mannosidase is essential for the maturation of glycoproteins which impacts cell surface signaling and protein stability. It also interacts with other enzymes and molecular chaperones during glycoprotein assembly.

Pathways

Alpha-Mannosidase plays a role in the glycosylation pathway and degradation of glycoproteins in the lysosome. It is involved in the N-glycan degradation pathway where its action precedes the function of other enzymes like beta-galactosidase and sialidase. Its function aligns with various glycosidase enzymes enabling the complete hydrolysis of oligosaccharides. This enzyme's work is connected directly to the processes influencing cellular homeostasis and turnover of glycoproteins.

Enzyme malfunctions can lead to alpha-Mannosidosis and other glycoprotein storage diseases. Alpha-Mannosidosis is characterized by a deficiency in this enzyme leading to the accumulation of mannose-rich oligosaccharides in cells causing symptoms like intellectual disability and skeletal abnormalities. The relationship between alpha-Mannosidase and lysosomal proteins such as lysosomal alpha-glucosidase highlights its role in the development of these conditions. Abnormalities associated with the enzyme's pathway can also affect cellular communication and immune system function marking its important role in health and disease.