Molecular Classification
Enzyme, Glycosidase, Hydrolase (EC 3.2.1.33)
Other Names
amylo-1,6-glucosidase, dextrin 6-α-D-glucosidase, amylopectin 1,6-glucosidase, dextrin-1,6-glucosidase, glycogen phosphorylase-limit dextrin α-1,6-glucohydrolase[2][6]
Disease Roles
Glycogen storage diseases (notably Glycogen Storage Disease type III, Cori disease, due to debranching enzyme deficiency)Metabolic disorders[5]Other (potential research in carbohydrate metabolism-related diseases)

Amylo-α-1,6-glucosidase Overview

Amylo-α-1,6-glucosidase is a hydrolase enzyme (EC 3.2.1.33) that specifically catalyzes the hydrolysis of α-1,6 branch linkages in glycogen phosphorylase-limit dextrin, a critical step in glycogen breakdown. This activity is an integral part of the glycogen debranching system, particularly in mammals, where the enzyme is coupled with transferase activity in a single polypeptide called glycogen debranching enzyme[2][6]. Deficiency of this enzymatic function results in abnormal glycogen metabolism, manifesting clinically as Cori disease (Glycogen Storage Disease type III)[5][6]. The enzyme is important for releasing glucose from branched glycogen during times of energy demand. There is no evidence of incorrectness in the target name, but confusion may arise as α-glucosidase (EC 3.2.1.20) is a more general term for enzymes hydrolyzing α-1,4-linkages, while amylo-α-1,6-glucosidase is responsible for α-1,6-branch hydrolysis specifically[6][2].

Mechanism of Action

Substrate hydrolysis (enzymatic cleavage of α-1,6 glycosidic bonds in glycogen limit dextrins)

Biological Functions

Glycogen debranching
Hydrolysis of α-1,6-glycosidic bonds[2][6][5]
Energy metabolism

Disease Associations

Glycogen storage diseases (notably Glycogen Storage Disease type III, Cori disease, due to debranching enzyme deficiency)
Metabolic disorders[5]
Other (potential research in carbohydrate metabolism-related diseases)

Safety Considerations

  • Therapeutic challenges mainly concern enzyme deficiency causing disease; enzyme replacement therapies for related but distinct enzymes may have immunogenicity or infusion reactions[5].
  • No direct small-molecule inhibitors are currently in use for this specific enzyme; off-target effects would be theoretical.

Interacting Drugs

There are no commonly approved direct drugs listed for amylo-α-1,6-glucosidase; most therapeutic focus is on lysosomal α-glucosidase (GAA) replacement (e.g., alglucosidase alfa/Myozyme for Pompe disease)[1][5].

Associated Biomarkers

Biomarker
Enzyme activity assays for debranching enzyme function (used in diagnostic panels for glycogen storage diseases)