In vitro establishment of human fibroblasts of lysosomal diseases, GM1-gangliosidosis and Sandhoff disease, by transformation with origin-minus SV40 DNA. Issue 3 (March 1985)

• Record Type: Journal Article
• Authors: Momoi, Takashi; Furuya, Tatsuko; Suzuki, Yoshiyuki; Sato, Hiroko; Yamaguchi, Nubuo
• Is Part Of: Bioscience reports. Volume 5:Issue 3(1985)
• Page Start: 267
• Page End: 273
• Publication Date: 1985-03
• Subjects: Molecular biology -- Periodicals; Cytology -- Periodicals; 572.8
• DOI: 10.1007/BF01119596
• Languages: English
• ISSNs: 0144-8463

Abstract

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.