Cell-autonomous death of cerebellar purkinje neurons with autophagy in Niemann-Pick type C disease.
Niemann-Pick type C is a neurodegenerative lysosomal storage disorder caused by mutations in either of two genes, npc1 and npc2. Cells lacking Npc1, which is a transmembrane protein related to the Hedgehog receptor Patched, or Npc2, which is a secreted cholesterol-binding protein, have aberrant orga...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Public Library of Science (PLoS)
2005-07-01
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| Series: | PLoS Genetics |
| Online Access: | http://europepmc.org/articles/PMC1183526?pdf=render |