𝔖 Bobbio Scriptorium
✦   LIBER   ✦

Tay-Sachs disease and normal cerebellar cells in culture: Elevated levels of lysosomal enzymes in Tay-Sachs disease cells

✍ Scribed by Dr. Linda M. Hoffman; Steven E. Brooks; Larry Schneck


Publisher
John Wiley and Sons
Year
1982
Tongue
English
Weight
342 KB
Volume
8
Category
Article
ISSN
0360-4012

No coin nor oath required. For personal study only.

✦ Synopsis


Abstract

Tay‐Sachs disease and normal fetal cerebellar cells were maintained in culture for up to four weeks. Elevated levels of the lysosomal enzymes hexosaminidase B, β‐galactosidase, and acid phosphatase were observed in the Tay‐Sachs cells in long term cultures although elevated lysosomal enzyme levels were not observed in the normal cultures. Similarly, elevated lysosomal enzyme activities were observed in Tay‐Sachs cultures which had been treated with Concanavalin A.


📜 SIMILAR VOLUMES


Long-term intracellular retention of hex
✍ Steven E. Brooks; Linda M. Hoffman; Daniel Amsterdam; Masazumi Adachi; Larry Sch 📂 Article 📅 1981 🏛 John Wiley and Sons 🌐 English ⚖ 493 KB 👁 1 views

## Abstract Enzyme‐replacement treatment for metabolic storage disorders has been widely studied using model cell culture systems. This study determines the long‐term fate of human hexosaminidase A supplied to Tay‐Sachs disease brain and lung cells. Hex A retention studies showed that the incorpora

Tay-sachs disease brain cells in culture
✍ Dr. Linda M. Hoffman; Steven E. Brooks; Daniel Amsterdam; John Oropello; Larry S 📂 Article 📅 1980 🏛 John Wiley and Sons 🌐 English ⚖ 324 KB 👁 1 views

## 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 place