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Different Trafficking Phenotypes of Niemann-Pick C1 Gene Mutations Correlate with Various Alterations in Lipid Storage, Membrane Composition and Miglustat Amenability

Niemann-Pick Type C (NPC) is an autosomal recessive lysosomal storage disease leading to progressive neurodegeneration. Mutations in the NPC1 gene, which accounts for 95% of the cases, lead to a defect in intra-lysosomal trafficking of cholesterol and an accumulation of storage material including ch...

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Vydáno v:Int J Mol Sci
Hlavní autoři: Brogden, Graham, Shammas, Hadeel, Walters, Friederike, Maalouf, Katia, Das, Anibh M., Naim, Hassan Y., Rizk, Sandra
Médium: Artigo
Jazyk:Inglês
Vydáno: MDPI 2020
Témata:
On-line přístup:https://ncbi.nlm.nih.gov/pmc/articles/PMC7139583/
https://ncbi.nlm.nih.gov/pubmed/32204338
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3390/ijms21062101
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