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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...
Autores principales: | Wiweger, Malgorzata, Majewski, Lukasz, Adamek-Urbanska, Dobrochna, Wasilewska, Iga, Kuznicki, Jacek |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8111220/ https://www.ncbi.nlm.nih.gov/pubmed/33986646 http://dx.doi.org/10.3389/fncel.2021.647860 |
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