Type C Niemann-Pick disease. Lysosomal accumulation and defective intracellular mobilization of low density lipoprotein cholesterol.

@article{Sokol1988TypeCN,
  title={Type C Niemann-Pick disease. Lysosomal accumulation and defective intracellular mobilization of low density lipoprotein cholesterol.},
  author={Joseph Sokol and Joan E. Blanchette-Mackie and Howard S. Kruth and Nancy K. Dwyer and Lynn M. Amende and Jean Debrohun Butler and Eve Robinson and Satyananda Patel and Roscoe O. Brady and Marcy Comly},
  journal={The Journal of biological chemistry},
  year={1988},
  volume={263 7},
  pages={3411-7}
}
The intracellular accumulation of unesterified cholesterol was examined during 24 h of low density lipoprotein (LDL) uptake in normal and Niemann-Pick C fibroblasts by fluorescence microscopy with filipin staining and immunocytochemistry. Perinuclear fluorescence derived from filipin-sterol complexes was observed in both normal and mutant cells by 2 h. This perinuclear cholesterol staining reached its peak in normal cells at 6 h. Subsequent development of fluorescence during the remaining 18 h… CONTINUE READING
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