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In vitro establishment of human fibroblasts of lysosomal diseases, GM1-gangliosidosis and Sandhoff disease, by transformation with origin-minus SV40 DNA

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Bioscience Reports

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.

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Authors and Affiliations

  1. Division of Chemical Pathology, National Center for Nervous, Mental and Muscular Disorders, 4-1-1 Ogawahigashi-machi, 187, Kodaira, Tokyo, Japan

    Takashi Momoi, Tatsuko Furuya & Yoshiyuki Suzuki

  2. Department of Pediatrics, Faculty of Medicine, The University of Tokyo, Bunkyo-ku, 113, Tokyo, Japan

    Yoshiyuki Suzuki

  3. The Institute of Medical Science, The University of Tokyo, 4-6-1 Minato-ku, 108, Tokyo, Japan

    Hiroko Sato & Nubuo Yamaguchi

Authors
  1. Takashi Momoi
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  2. Tatsuko Furuya
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  3. Yoshiyuki Suzuki
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  4. Hiroko Sato
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  5. Nubuo Yamaguchi
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Momoi, T., Furuya, T., Suzuki, Y. et al. In vitro establishment of human fibroblasts of lysosomal diseases, GM1-gangliosidosis and Sandhoff disease, by transformation with origin-minus SV40 DNA. Biosci Rep 5, 267–273 (1985). https://doi.org/10.1007/BF01119596

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  • DOI: https://doi.org/10.1007/BF01119596

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