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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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| Autori principali: | , , , , |
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| Natura: | Artigo |
| Lingua: | Inglês |
| Pubblicazione: |
Oxford University Press
2014
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| Soggetti: | |
| Accesso online: | 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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