Debranchingentsyymi
Debranchingentsyymi is a cytosolic enzyme involved in the breakdown of glycogen and other branched glucose polymers. It functions as part of the glycogenolysis pathway, acting after glycogen phosphorylase has removed many glucose units from the ends of chains. Its action allows complete proteolysis of branched structures rather than leaving inaccessible limit branches.
The enzyme carries two coordinated activities. First, a transferase activity (4-α-glucanotransferase) shifts a short chain of
Genetics and distribution: In humans, the debranching enzyme is encoded by the AGL gene and is found
Clinical relevance: Deficiency of debranching functionality leads to glycogen storage disease type III, also called Cori
Structure and regulation: Debranchingentsyymi comprises domains corresponding to its transferase and glucosidase activities within a single