Cessation of rapid late endosomal tubulovesicular trafficking in Niemann-Pick type C1 disease.

@article{Zhang2001CessationOR,
  title={Cessation of rapid late endosomal tubulovesicular trafficking in Niemann-Pick type C1 disease.},
  author={Mingdong Zhang and Nancy K. Dwyer and Dona C. Love and Adele M. Cooney and Marcy Comly and Edward B. Neufeld and Peter G. Pentchev and E. Joan Blanchette-Mackie and John A Hanover},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  year={2001},
  volume={98 8},
  pages={
          4466-71
        }
}
Niemann-Pick type C1 (NPC1) disease results from a defect in the NPC1 protein and is characterized by a pathological accumulation of cholesterol and glycolipids in endocytic organelles. We followed the biosynthesis and trafficking of NPC1 with the use of a functional green fluorescent protein-fused NPC1. Newly synthesized NPC1 is exported from the endoplasmic reticulum and requires transit through the Golgi before it is targeted to late endosomes. NPC1-containing late endosomes then move by a… CONTINUE READING

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