Anti-Galactosidase alpha antibody [EPR23303-249] - BSA and Azide free (Detector)

Supplementary info

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

Activity summary

Alpha-galactosidase also known as alpha-D-galactosidase or A-galactosidase is an enzyme that catalyzes the hydrolysis of terminal alpha-galactosyl moieties from glycolipids and glycoproteins. It has a molecular mass of approximately 50000 Daltons. This enzyme is expressed in various tissues including the liver heart and kidneys. It functions by efficiently removing alpha-galactose residues from target molecules a process important for maintaining cellular function.

Biological function summary

The role of alpha-galactosidase involves the breakdown of complex carbohydrates particularly those containing alpha-galactosidic bonds. It does not operate as part of a complex but rather functions independently to cleave these specific bonds in glycolipids and glycoproteins. This activity is critical for degrading langerin and other gangliosides preventing the accumulation of these molecules in the lysosomes. By continuously performing this function alpha-galactosidase maintains glycosphingolipid homeostasis ensuring efficient cellular metabolism.

Pathways

The involvement of alpha-galactosidase in metabolic and lysosomal catabolic pathways is significant. One critical pathway is the glycolipid metabolism pathway where it acts alongside related enzymes such as beta-galactosidase. It also participates in the lysosomal degradation pathway working in conjunction with other lysosomal enzymes to prevent substrate buildup. The interaction with enzymes like lysosomal hydrolases ensures the proper breakdown of complex molecules into simpler ones that cells can utilize or excrete.

Associated diseases and disorders

Defects in the alpha-galactosidase enzyme lead to the lysosomal storage disorder known as Fabry disease. This genetic disorder causes the accumulation of globotriaosylceramide due to deficient alpha-galactosidase activity. Another disorder associated with malfunctioning alpha-galactosidase is cardiac complications due to tissue storage of glycolipids. In both cases the lack of enzyme activity disrupts cellular and tissue functions linking alpha-galactosidase to clinical symptoms seen in these disorders.