Please use this identifier to cite or link to this item: http://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
RMID: 0020061180
DOI: 10.1016/j.febslet.2006.06.096
Appears in Collections:Paediatrics publications

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