Glycogen limit dextrin

Glycogen limit dextrin is a branched polysaccharide intermediate formed during the intracellular degradation of glycogen. It is produced when the enzyme glycogen phosphorylase cleaves alpha(1->4) glycosidic bonds but stops four glucose residues away from an alpha(1->6) branch point, as the enzyme's active site cannot accommodate the branched structure (Berg et al., 2002 [1]). Further degradation requires the bifunctional glycogen debranching enzyme (GDE), which relocates the outer three residues of the branch to a nearby linear chain and then hydrolyzes the remaining alpha(1->6) linkage (StatPearls, 2023 [2]). In Glycogen Storage Disease Type III (GSD III), also known as Cori or Forbes disease, a deficiency in GDE leads to the pathological accumulation of limit dextrin in the liver, heart, and skeletal muscles (NIH, 2020 [3]). This accumulation is a hallmark of the disease and leads to symptoms such as hepatomegaly, growth retardation, and myogenic damage (PubMed, 2018 [4]). While the molecule itself is not a target for traditional drug binding, therapeutic strategies focus on managing its accumulation through dietary interventions or investigating enzyme replacement and gene therapies (Journal of Inherited Metabolic Disease, 2021 [5]).

Other names

Limit dextrinPhosphorylase limit dextrinGlycogen phosphorylase limit dextrin