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npc2-Deficient Zebrafish Reproduce Neurological and Inflammatory Symptoms of Niemann-Pick Type C Disease

Niemann-Pick type C (NPC) disease is an autosomal recessive lysosomal storage disease that is caused by a mutation of the NPC1 or NPC2 gene, in which un-esterified cholesterol and sphingolipids accumulate mainly in the liver, spleen, and brain. Abnormal lysosomal storage leads to cell damage, neurol...

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Principais autores: Malgorzata Wiweger, Lukasz Majewski, Dobrochna Adamek-Urbanska, Iga Wasilewska, Jacek Kuznicki
Formato: Artigo
Idioma:Inglês
Publicado em: Frontiers Media S.A. 2021-04-01
coleção:Frontiers in Cellular Neuroscience
Assuntos:
Niemann-Pick type C
npc2
zebrafish model
Nile blue
lysosomal storage disease
neurodegeneration
Acesso em linha:https://www.frontiersin.org/articles/10.3389/fncel.2021.647860/full
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