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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...

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Detalles Bibliográficos
Autores principales: Timur, Z.K., Akyildiz Demir, S., Marsching, C., Sandhoff, R., Seyrantepe, V.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2015
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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