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Reversibility of neuropathology in Tay–Sachs-related diseases

The GM2 gangliosidoses are progressive neurodegenerative disorders due to defects in the lysosomal β-N-acetylhexosaminidase system. Accumulation of β-hexosaminidases A and B substrates is presumed to cause this fatal condition. An authentic mouse model of Sandhoff disease (SD) with pathological char...

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Main Authors: Cachón-González, María-Begoña, Wang, Susan Z., Ziegler, Robin, Cheng, Seng H., Cox, Timothy M.
Formato: Artigo
Idioma:Inglês
Publicado: Oxford University Press 2014
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Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC3888261/
https://ncbi.nlm.nih.gov/pubmed/24057669
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddt459
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