Characterization of residual hexosaminidase activity in Sandhoff's disease using man-Chinese hamster cell hybrids
✍ Scribed by H. L. Hoeksema; A. J. J. Reuser; A. T. Hoogeveen; A. Westerveld; H. Galjaard
- Publisher
- Springer
- Year
- 1977
- Tongue
- English
- Weight
- 400 KB
- Volume
- 39
- Category
- Article
- ISSN
- 0340-6717
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✦ Synopsis
To obtain information about the nature of the residual hexosaminidase activity in Sandhoff's disease, hybrid cell lines between fibroblasts from a patient with Sandhoff's disease and Chinese hamster cells were isolated. In these hybrid cell lines, a heteropolymeric isoenzyme was detected that is composed of human alpha- and Chinese hamster hexosaminidase subunits. Due to the electrophoretic and immunological behavior of the heteropolymeric molecules in interspecies hybrids with normal fibroblasts and with cells from a patient with Sandhoff's disease, we conclude that Sandhoff cells contain an alpha-subunit of hexosaminidase with normal characteristics.