Embryonic striatal neurons from niemann-pick type C mice exhibit defects in cholesterol metabolism and neurotrophin responsiveness.

@article{Henderson2000EmbryonicSN,
  title={Embryonic striatal neurons from niemann-pick type C mice exhibit defects in cholesterol metabolism and neurotrophin responsiveness.},
  author={Leslie P. Henderson and Lih-Ling Lin and Aparna Prasad and Colleen A Paul and T. Y. P. Chang and Robert A. Maue},
  journal={The Journal of biological chemistry},
  year={2000},
  volume={275 26},
  pages={20179-87}
}
Niemann-Pick type C (NP-C) disease is a progressive and fatal neuropathological disorder previously characterized by abnormal cholesterol metabolism in peripheral tissues. Although a defective gene has been identified in both humans and the npc(nih) mouse model of NP-C disease, how this leads to abnormal neuronal function is unclear. Here we show that whereas embryonic striatal neurons from npc(nih) mice can take up low density lipoprotein-derived cholesterol, its subsequent hydrolysis and… CONTINUE READING

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Neuronal Defects in NP-C Mice 20186 by gest on D ecem er 24

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