Membrane traffic in sphingolipid storage diseases.

  title={Membrane traffic in sphingolipid storage diseases.},
  author={Richard E. Pagano and Vinita Puri and Michel Dom{\'i}nguez and David L. Marks},
  volume={1 11},
In this review, we summarize our studies of membrane lipid transport in sphingolipid storage disease (SLSD) fibroblasts. We recently showed that several fluorescent SL analogs were internalized from the plasma membrane predominantly to the Golgi complex of normal cells, while in ten different SLSD cell types, these lipids accumulated in endosomes and lysosomes (The Lancet 1999;354: 901-905). Additional studies showed that cholesterol homeostasis is perturbed in multiple SLSDs secondary to SL… CONTINUE READING