Alexa Fluor® 568 Anti-AGL/Alpha-glucosidase antibody [EPR8880]

Supplementary info

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

Activity summary

Alpha-glucosidase also known as AGL is an enzyme that breaks down carbohydrates by hydrolyzing alpha-14 glycosidic bonds in glycogen converting them into glucose molecules. This enzyme is important for the body's ability to derive glucose from stored glycogen a necessary process for maintaining energy levels. AGL is predominantly expressed in liver tissue where it plays an important role in glycogen metabolism. The AGL enzyme has an approximate mass of around 104 kDa. Additionally AGL can be referred to as alpha-16-glucosidase due to its specific hydrolytic capabilities on branching points in glycogen.

Biological function summary

AGL functions as part of the glycogen debranching enzyme complex which is important for glycogen catabolism. It cooperates with other enzymes to effectively mobilize glucose from glycogen stores. AGL's debranching activity ensures the smooth continuation of glycogenolysis providing glucose to meet energy demands during fasting or intense physical activity. This highlights the enzyme's importance in glucose homeostasis and metabolic processes that require rapid energy availability.

Pathways

AGL participates in the glycogenolysis and gluconeogenesis pathways. It partners with phosphorylase to release glucose-1-phosphate from glycogen ensuring energy supply during fasting conditions. The glycogenolysis pathway relies significantly on AGL for breaking down glycogen's branched structures while glucose-6-phosphatase assists in converting glucose-6-phosphate into free glucose. AGL's role in these pathways illustrates its importance in energy metabolism and glucose regulation.

Associated diseases and disorders

AGL's dysfunction is linked to glycogen storage disease type III known as Cori disease. This genetic disorder results from mutations in the AGL gene causing a build-up of abnormally structured glycogen in the liver and muscles leading to symptoms such as hepatomegaly muscular weakness and hypoglycemia. AGL along with other enzymes involved in glycogen metabolism like phosphorylase kinase is a target for therapeutic interventions aimed at managing such glycogen storage diseases.