Article
[Change of isoforms' spectrum of alpha-L-fucoside secreted by affected cells in some hereditary lysosomal diseases].
Voprosy meditsinskoi khimii - 1 Jan 2000
Ivleva T S, Beíer E M, Ushakova N A, Malakhova V A, Karpova E A, Tsvetkova I V
Abstract excerpt
In vitro it was studied the isoform spectra of the intracellular and secreted alpha-L-fucosidase from skin fibroblasts of patients with Fabry disease (glycolipidosis), Hurler and Sanfilippo D diseases (mucopolysaccharodosis, types I and III) and in the normal state was studied. It was shown that the multiple form profile of secreted alpha-L-fucosidase in patients fibroblasts was changed as compared to that in...
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