Free sphingoid bases in tissues from patients with type C Niemann-Pick disease and other lysosomal storage disorders.

@article{RodriguezLafrasse1994FreeSB,
  title={Free sphingoid bases in tissues from patients with type C Niemann-Pick disease and other lysosomal storage disorders.},
  author={Claire Rodriguez-Lafrasse and Robert Rousson and Peter G. Pentchev and Pierre Louisot and Marie Th{\'e}r{\`e}se Vanier},
  journal={Biochimica et biophysica acta},
  year={1994},
  volume={1226 2},
  pages={138-44}
}
The 20-fold increase of free sphingoid bases found in liver from a murine model of Niemann-Pick type C (NPC) combined to the NPC-like phenotype induced by addition of sphinganine to normal fibroblast cultures prompted us to investigate the potential involvement of these compounds in the human disease. The contents of sphingosine and sphinganine were measured in liver, spleen, brain and skin fibroblast cultures by a sensitive HPLC method. In liver and spleen from NPC patients, a 6- to 24-fold… CONTINUE READING

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