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|Title:||Stabilising normal and mis-sense variant α-glucosidase|
|Other Titles:||Stabilising normal and mis-sense variant alpha-glucosidase|
|Citation:||FEBS Letters, 2006; 580(18):4365-4370|
|Publisher:||Elsevier Science BV|
|Kakavanos, Revecca ; Hopwood, John J. ; Lang, Debbie ; Meikle, Peter J. ; Brooks, Doug A.|
|Abstract:||alpha-Glucosidase (EC 126.96.36.199) 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 residual level of alpha-glucosidase activity, suggesting that a structural analogue of d-glucose may be used for enzyme enhancement therapy.|
|Keywords:||α-Glucosidase; Enzyme stability; Pompe disease; Enzyme enhancement|
|Appears in Collections:||Paediatrics publications|
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