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L-Arginine Ameliorates Defective Autophagy in GM2 Gangliosidoses by mTOR Modulation
Aims: Tay–Sachs and Sandhoff diseases (GM2 gangliosidosis) are autosomal recessive disorders of lysosomal function that cause progressive neurodegeneration in infants and young children. Impaired hydrolysis catalysed by β-hexosaminidase A (HexA) leads to the accumulation of GM2 ganglioside in neuron...
Autores principales: | Castejón-Vega, Beatriz, Rubio, Alejandro, Pérez-Pulido, Antonio J., Quiles, José L., Lane, Jon D., Fernández-Domínguez, Beatriz, Cachón-González, María Begoña, Martín-Ruiz, Carmen, Sanz, Alberto, Cox, Timothy M., Alcocer-Gómez, Elísabet, Cordero, Mario D. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8619250/ https://www.ncbi.nlm.nih.gov/pubmed/34831346 http://dx.doi.org/10.3390/cells10113122 |
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