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Tay‐sachs disease brain cells in culture: Mobilization of stored G M2 after concanavalin A‐mediated uptake of hexosaminidase A
Author(s) -
Hoffman Linda M.,
Brooks Steven E.,
Amsterdam Daniel,
Oropello John,
Schneck Larry
Publication year - 1980
Publication title -
journal of neuroscience research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.72
H-Index - 160
eISSN - 1097-4547
pISSN - 0360-4012
DOI - 10.1002/jnr.490050506
Subject(s) - hexosaminidase , concanavalin a , ganglioside , cell culture , biology , microbiology and biotechnology , biochemistry , chemistry , in vitro , enzyme , genetics
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.