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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| Authors: | Takashi Momoi Tatsuko Furuya Yoshiyuki Suzuki Hiroko Sato Nubuo Yamaguchi |
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| Institution: | (1) Division of Chemical Pathology, National Center for Nervous, Mental and Muscular Disorders, 4-1-1 Ogawahigashi-machi, 187 Kodaira, Tokyo, Japan;(2) Department of Pediatrics, Faculty of Medicine, The University of Tokyo, Bunkyo-ku, 113 Tokyo, Japan;(3) The Institute of Medical Science, The University of Tokyo, 4-6-1 Minato-ku, 108 Tokyo, Japan |
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| 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  -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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