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Neuronal network dysfunction precedes storage and neurodegeneration in a lysosomal storage disorder
Accumulation of lysosomal storage material and late-stage neurodegeneration are hallmarks of lysosomal storage disorders (LSDs) affecting the brain. Yet, for most LSDs, including CLN3 disease, the most common form of childhood dementia, it is unclear what mechanisms drive neurologic symptoms. Do def...
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| Publicado no: | JCI Insight |
|---|---|
| Main Authors: | , , , , , , |
| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
American Society for Clinical Investigation
2019
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| Assuntos: | |
| Acesso em linha: | https://ncbi.nlm.nih.gov/pmc/articles/PMC6948765/ https://ncbi.nlm.nih.gov/pubmed/31573978 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/jci.insight.131961 |
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