Tay-sachs disease brain cells in culture: Mobilization of stored GM2 after concanavalin A-mediated uptake of hexosaminidase A

✍ Scribed by Dr. Linda M. Hoffman; Steven E. Brooks; Daniel Amsterdam; John Oropello; Larry Schneck

• Publisher: John Wiley and Sons
• Year: 1980
• Tongue: English
• Weight: 324 KB
• Volume: 5
• Category: Article
• ISSN: 0360-4012
• DOI: 10.1002/jnr.490050506

✦ Synopsis

Abstract

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.