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A mutation in the ovine cathepsin D gene causes a congenital lysosomal storage disease with profound neurodegeneration

The neuronal ceroid lipofuscinoses (NCLs) constitute a group of neurodegenerative storage diseases characterized by progressive psychomotor retardation, blindness and premature death. Pathologically, there is accumulation of autofluorescent material in lysosome-derived organelles in a variety of cel...

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Detalhes bibliográficos
Main Authors: Tyynelä, Jaana, Sohar, Istvan, Sleat, David E., Gin, Rosalie M., Donnelly, Robert J., Baumann, Marc, Haltia, Matti, Lobel, Peter
Formato: Artigo
Idioma:Inglês
Publicado em: Oxford University Press 2000
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC203370/
https://ncbi.nlm.nih.gov/pubmed/10856224
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/emboj/19.12.2786
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