Tay‐sachs disease brain cells in culture: Mobilization of stored G M2 after concanavalin A‐mediated uptake of hexosaminidase A

A human Tay‐Sachs disease (TSD) fetal‐brain‐cell line is a useful model for the disease since the cells lack hexosaminidase A and accumulate the ganglioside, G M2 . This brain‐cell line was used to assess the effect of hexosaminidase A treatment on G M2 storage material. Entry of placental hexosaminidase A into the cells was obtained by pretreatment of the cultures with concanavalin A. Cells were analyzed periodically during six days. During the course of the experiment, G M2 in the cells decreased by approximately 50%. A substantial amount of hexosaminidase A was maintained in the cultures throughout the experiment. This strategy was successful in mobilizing stored G M2 in TSD brain‐cell cultures. Therefore, the activating factor needed for hexosaminidase A activity must be present in TSD‐cultured brain cells.