Natural history study of glycan accumulation in large animal models of GM2 gangliosidoses.
β-hexosaminidase is an enzyme responsible for the degradation of gangliosides, glycans, and other glycoconjugates containing β-linked hexosamines that enter the lysosome. GM2 gangliosidoses, such as Tay-Sachs and Sandhoff, are lysosomal storage disorders characterized by β-hexosaminidase deficiency...
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| Autores principales: | , , , , , , , , |
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| Formato: | Artigo |
| Lenguaje: | Inglês |
| Publicado: |
Public Library of Science (PLoS)
2020-01-01
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| Colección: | PLoS ONE |
| Acceso en línea: | https://doi.org/10.1371/journal.pone.0243006 |
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