Characterization of residual hexosaminidase activity in Sandhoff's disease using man-Chinese hamster cell hybrids

Summary To obtain information about the nature of the residual hexosaminidase activity in Sandhoff's disease, hybrid cell lines between fibroblasts from a patients 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 - 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 -subunit of hexosaminidase with normal characteristics.