Amylo-1,6-glucosidase

Amylo-1 —> 6-glucosidase obtained by Cori and Larner218 from rabbit muscles, and R-enzyme isolated by Hobson, Whelan and Peat219 from potatoes and broad beans, are typical debranching enzymes, which will hydrolyze the 6 — 1-a-D-glucosidic linkage rather than the normal 4 —> 1-a-D linkage. These enzymes will therefore be particularly important in determinations of the fine structure of amylopectin, if they can be sufficiently well purified. 

Table 4.6.11 Mixtures for liquid scintillation counting of amylo-1,6-glucosidase (debranching enzyme assay with [14C]-glucose) for liver and muscle, and for RBC...

Amylo-(1 6)-glucosidase is the a-D-(l— 6)-debranching enzyme found in animal skeletal muscle, where it debranches glycogen. Methods of preparation have been described, but there is no knowledge regarding its structure or physical properties. 

III Cori Amylo-1,6 glucosidase debranching enzyme Liver, muscle, WBCs... 

III Limit dextrinosis 30 liver, muscle limit dextrin amylo-1,6-glucosidase... 

A polysaccharide having single-unit A-chains is the logical substrate for amylo-1,6-glucosidase whether this is the true ( >-dextrin or a modified... 

In the relative absence of amylo-1,6-glucosidase, as in Type III glycogen-storage disease, glycogen breakdown is incomplete and is limited to the exterior chains. The enzyme therefore fulfils an important in vivo role. 

III Amylo-1,6-glucosidase (deb rancher) Liver, skeletal muscle, heart Fasting hypoglycemia hepatomegaly in infancy in some, myopathic features. Glycogen deposits have short outer branches. 

III. Cori Amylo-1,6-glucosidase (debranching enzyme) Muscle and liver Increased amount short oirter branches Hepatomegaly, less severe hypoglycemia but may have more severe ketoacidosis without high lactate or uric acid. Increased AST normal blood lactate and uric acid... 

---