The expression of hex A and hex B isozymes of hexosaminidase in parental and experimental human fibroblast cells and their components.

The expression of the two major isozyme forms of hexosaminidase (EC 3.2.1.30), hexosaminidase A and hexosaminidase B, has been examined. The parental cells and/or cellular components of parental cells are individually fused using inactivated Sendai virus with the aid of a micromanipulator. The progeny cells produced from such hybrids are subjected to a microenzymatic assay which allows measurements at the single cell level. The lysosomal-deficient cells used in this study are Tay-Sachs and Sandhoff fibroblasts, and the normal cells used are WI-38 (fetal lung fibroblasts), amniotic fluid cells (GM 473), and JASD3 (normal human foreskin). The results show that the ratio of cell components which are fused to form the experimental cell affects the percentage of hexosaminidase A expressed in the progeny cells. Furthermore, our results imply the presence of a "factor" in the Sandhoff cell's cytoplasm which, together with the Tay-Sachs nucleus, is necessary for hexosaminidase A expression in the experimental cell's progeny.