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https://hdl.handle.net/2440/23281
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Type: | Journal article |
Title: | Stabilising normal and mis-sense variant α-glucosidase |
Other Titles: | Stabilising normal and mis-sense variant alpha-glucosidase |
Author: | Kakavanos, R. Hopwood, J. Lang, D. Meikle, P. Brooks, D. |
Citation: | FEBS Letters, 2006; 580(18):4365-4370 |
Publisher: | Elsevier Science BV |
Issue Date: | 2006 |
ISSN: | 0014-5793 1873-3468 |
Statement of Responsibility: | Kakavanos, Revecca ; Hopwood, John J. ; Lang, Debbie ; Meikle, Peter J. ; Brooks, Doug A. |
Abstract: | alpha-Glucosidase (EC 3.2.1.3) 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 |
DOI: | 10.1016/j.febslet.2006.06.096 |
Published version: | http://dx.doi.org/10.1016/j.febslet.2006.06.096 |
Appears in Collections: | Aurora harvest 6 Paediatrics publications |
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