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Lysosomal storage of heparan sulfate causes mitochondrial defects, altered autophagy, and neuronal death in the mouse model of mucopolysaccharidosis III type C

The genetic metabolic disease mucopolysaccharidosis III type C (MPS IIIC, Sanfilippo disease type C) causes progressive neurodegeneration in infants and children, leading to dementia and death before adulthood. MPS IIIC stands out among lysosomal diseases because it is the only one caused by a defic...

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Detalhes bibliográficos
Publicado no:Autophagy
Autor principal: Pshezhetsky, Alexey V.
Formato: Artigo
Idioma:Inglês
Publicado em: Taylor & Francis 2015
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4922431/
https://ncbi.nlm.nih.gov/pubmed/25998837
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1080/15548627.2015.1046671
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