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Intracellular Accumulation of Amyloidogenic Fragments of Amyloid-β Precursor Protein in Neurons with Niemann-Pick Type C Defects Is Associated with Endosomal Abnormalities

Niemann-Pick type C disease (NPC) is characterized by neurodegeneration secondary to impaired cholesterol trafficking and excessive glycosphingolipid storage. Abnormal cholesterol and ganglioside metabolism may influence the generation and aggregation of amyloidogenic fragments (ie, C99 and Aβ) from...

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Autori principali: Jin, Lee-Way, Maezawa, Izumi, Vincent, Inez, Bird, Thomas
Natura: Artigo
Lingua:Inglês
Pubblicazione: American Society for Investigative Pathology 2004
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC1614713/
https://ncbi.nlm.nih.gov/pubmed/14982851
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