ISSN:
1432-0533
Keywords:
Enzyme replacement
;
Hex A
;
TSD
;
Con A
;
Brain cells
;
GM2
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary When Concanavalin A (Con A) is bound to the cell membrane, it functions as an artificial enzyme receptor, mediating the binding and intracellular incorporation of significant amounts of exogenous hexosaminidase A (Hex A) into Tay-Sachs disease (TSD) glial cells. The treated cells retained almost 50% of incorporated Hex A activity after 3 days incubation in Hex A free medium. Hex A was released from Con A within the cell and was available as free enzyme. Biochemical analysis of gangliosides in Con A and Hex A treated cells depicted a greater than 50% reduction in stored GM2 ganglioside and a fourfold reduction in GM2label (14C) when compared to controls. Ultrastructural evidence of GM2 breakdown is presented which supports the biochemical and labeling studies.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00688529
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