Characterization of residual hexosaminidase activity in Sandhoff's disease using man-Chinese hamster cell hybrids.
To obtain information about the nature of the residual hexosaminidase activity in Sandhoff's disease, hybrid cell lines between fibroblasts from a patient with Sandhoff's disease and Chinese hamster cells were isolated. In these hybrid cell lines, a heteropolymeric isoenzyme was detected that is composed of human alpha- and Chinese hamster hexosaminidase subunits. Due to the electrophoretic and immunological behavior of the heteropolymeric molecules in interspecies hybrids with normal fibroblasts and with cells from a patient with Sandhoff's disease, we conclude that Sandhoff cells contain an alpha-subunit of hexosaminidase with normal characteristics.
D000818:Animals; D006224:Cricetinae; D005347:Fibroblasts / Q000201:enzymology; D005733:Gangliosidoses / Q000201:enzymology*; D006596:Hexosaminidases / Q000032:analysis*; D006801:Humans; D006822:Hybrid Cells / Q000201:enzymology; D007527:Isoenzymes / Q000032:analysis
D007527:Isoenzymes; D006596:Hexosaminidases