Group C Niemann-Pick disease: faulty regulation of low-density lipoprotein uptake and cholesterol storage in cultured fibroblasts.

@article{Pentchev1987GroupCN,
  title={Group C Niemann-Pick disease: faulty regulation of low-density lipoprotein uptake and cholesterol storage in cultured fibroblasts.},
  author={Peter G. Pentchev and Marcy Comly and Howard S. Kruth and Toshiko Tokoro and Jean Debrohun Butler and Josh Sokol and M R Filling-Katz and Jane M. Quirk and Dawn C. Marshall and Shailja Patel},
  journal={FASEB journal : official publication of the Federation of American Societies for Experimental Biology},
  year={1987},
  volume={1 1},
  pages={40-5}
}
Incubation of mutant Niemann-Pick C fibroblasts with low-density lipoprotein (LDL) resulted in excessive internalization of lipoprotein and extensive cellular over-accumulation of unesterified cholesterol. The uptake of LDL by the mutant cells appeared to occur through the classic LDL receptor pathway and internalized lipoprotein was processed in lysosomes. Lipoprotein uptake into mutant cells was associated with delays in the initiation of established cellular cholesterol homeostatic responses… CONTINUE READING