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...
Main Authors: | Beatriz Castejón-Vega, Alejandro Rubio, Antonio J. Pérez-Pulido, José L. Quiles, Jon D. Lane, Beatriz Fernández-Domínguez, María Begoña Cachón-González, Carmen Martín-Ruiz, Alberto Sanz, Timothy M. Cox, Elísabet Alcocer-Gómez, Mario D. Cordero |
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Format: | Article |
Language: | English |
Published: |
MDPI AG
2021-11-01
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Series: | Cells |
Subjects: | |
Online Access: | https://www.mdpi.com/2073-4409/10/11/3122 |
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