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https://hdl.handle.net/2440/23281
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DC Field | Value | Language |
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dc.contributor.author | Kakavanos, R. | - |
dc.contributor.author | Hopwood, J. | - |
dc.contributor.author | Lang, D. | - |
dc.contributor.author | Meikle, P. | - |
dc.contributor.author | Brooks, D. | - |
dc.date.issued | 2006 | - |
dc.identifier.citation | FEBS Letters, 2006; 580(18):4365-4370 | - |
dc.identifier.issn | 0014-5793 | - |
dc.identifier.issn | 1873-3468 | - |
dc.identifier.uri | http://hdl.handle.net/2440/23281 | - |
dc.description.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. | - |
dc.description.statementofresponsibility | Kakavanos, Revecca ; Hopwood, John J. ; Lang, Debbie ; Meikle, Peter J. ; Brooks, Doug A. | - |
dc.language.iso | en | - |
dc.publisher | Elsevier Science BV | - |
dc.source.uri | http://dx.doi.org/10.1016/j.febslet.2006.06.096 | - |
dc.subject | α-Glucosidase | - |
dc.subject | Enzyme stability | - |
dc.subject | Pompe disease | - |
dc.subject | Enzyme enhancement | - |
dc.title | Stabilising normal and mis-sense variant α-glucosidase | - |
dc.title.alternative | Stabilising normal and mis-sense variant alpha-glucosidase | - |
dc.type | Journal article | - |
dc.identifier.doi | 10.1016/j.febslet.2006.06.096 | - |
pubs.publication-status | Published | - |
dc.identifier.orcid | Brooks, D. [0000-0001-9098-3626] | - |
Appears in Collections: | Aurora harvest 6 Paediatrics publications |
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