In vitro establishment of human fibroblasts of lysosomal diseases, GM1-gangliosidosis and Sandhoff disease, by transformation with origin-minus SV40 DNA
- 1 March 1985
- journal article
- Published by Portland Press Ltd. in Bioscience Reports
- Vol. 5 (3) , 267-273
- https://doi.org/10.1007/bf01119596
Abstract
The permanent human cell lines preserving defects of lysosomal enzymes, GM1-1019-SV and SA-1077-SV, were established from the respective fibroblasts from patients with GMl-gangliosidosis and Sandhoff disease by transfection with replication origin-minus simian virus 40 DNA. These ceils grow rapidly without entering senescence during more than 120 population doublings. The activity of β-galactosidase in GM1-1019-SV and of B-N-acetylhexosaminidase in SA-1077-SV was respectively 40- and 180-fold lower than that of normal fibroblasts.Keywords
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