Assay of ganglioside GM2-N-acetyl-beta-D-galactosaminidase activity in human fibroblasts employing the natural activator protein--diagnosis of variant forms of GM2 gangliosidosis.

@article{Erzberger1980AssayOG,
  title={Assay of ganglioside GM2-N-acetyl-beta-D-galactosaminidase activity in human fibroblasts employing the natural activator protein--diagnosis of variant forms of GM2 gangliosidosis.},
  author={A Erzberger and Ernst Conzelmann and Konrad Sandhoff},
  journal={Clinica chimica acta; international journal of clinical chemistry},
  year={1980},
  volume={108 3},
  pages={
          361-8
        }
}
The physiological activator protein for the degradation of ganglioside GM2 by hexosaminidase A has been employed to assess the capability of cultured human fibroblast extracts to catalyze this ganglioside. This method permits a more reliable diagnosis of the different variants of GM2 gangliosidoses than the methods hitherto used. These either rely on artificial substrates or, when natural substrates are used, on detergents. Our method avoids a number of possible sources of error introduced by… CONTINUE READING
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