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Neuraminidase-1 contributes significantly to the degradation of neuronal B-series gangliosides but not to the bypass of the catabolic block in Tay–Sachs mouse models
Tay–Sachs disease is a severe lysosomal storage disorder caused by mutations in the HEXA gene coding for α subunit of lysosomal β-Hexosaminidase A enzyme, which converts G(M2) to G(M3) ganglioside. HexA(−/−) mice, depleted of the β-Hexosaminidase A iso-enzyme, remain asymptomatic up to 1 year of age...
Autores principales: | Timur, Z.K., Akyildiz Demir, S., Marsching, C., Sandhoff, R., Seyrantepe, V. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4750590/ https://www.ncbi.nlm.nih.gov/pubmed/26937414 http://dx.doi.org/10.1016/j.ymgmr.2015.07.004 |
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