Journal article

Stabilising normal and mis-sense variant alpha-glucosidase

Revecca Kakavanos, John J Hopwood, Debbie Lang, Peter J Meikle, Doug A Brooks

FEBS Letters | WILEY | Published : 2006


alpha-Glucosidase (EC is a lysosomal enzyme that hydrolyses alpha-1,4- and alpha-1,6-linkages of glycogen to produce free glucose. A deficiency in alpha-glucosidase activity results in glycogen storage disorder type II (GSD II), also called Pompe disease. Here, d-glucose was shown to be a competitive inhibitor of alpha-glucosidase and when added to culture medium at 6.0 g/L increased the production of this protein by CHO-K1 expression cells and stabilised the enzyme activity. D-Glucose also prevented alpha-glucosidase aggregation/precipitation and increased protein yield in a modified purification scheme. In fibroblast cells, from adult-onset GSD II patients, D-glucose increased the..

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