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Comparative proteomic profiling reveals mechanisms for early spinal cord vulnerability in CLN1 disease

CLN1 disease is a fatal inherited neurodegenerative lysosomal storage disease of early childhood, caused by mutations in the CLN1 gene, which encodes the enzyme Palmitoyl protein thioesterase-1 (PPT-1). We recently found significant spinal pathology in Ppt1-deficient (Ppt1(−/−)) mice and human CLN1...

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Detalles Bibliográficos
Autores principales:	Nelvagal, Hemanth R., Hurtado, Maica Llavero, Eaton, Samantha L., Kline, Rachel A., Lamont, Douglas J., Sands, Mark S., Wishart, Thomas M., Cooper, Jonathan D.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Nature Publishing Group UK 2020
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7495486/ https://www.ncbi.nlm.nih.gov/pubmed/32938982 http://dx.doi.org/10.1038/s41598-020-72075-7

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