Increased Activity and Altered Subcellular Distribution of Lysosomal Enzymes Determine Neuronal Vulnerability in Niemann-Pick Type C1-Deficient Mice

Niemann-Pick disease type C (NPC), caused by mutations in the Npc1 or Npc2 genes, is a progressive neurodegenerative disorder characterized by intracellular accumulation/redistribution of cholesterol in a number of tissues including the brain. This is accompanied by a severe loss of neurons in selec...

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Hauptverfasser: Amritraj, Asha, Peake, Kyle, Kodam, Anitha, Salio, Chiara, Merighi, Adalberto, Vance, Jean E., Kar, Satyabrata
Format: Artigo
Sprache:Inglês
Veröffentlicht: American Society for Investigative Pathology 2009
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Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC2789601/
https://ncbi.nlm.nih.gov/pubmed/19893049
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.2353/ajpath.2009.081096
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