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Article

L-Arginine Ameliorates Defective Autophagy in GM2 Gangliosidoses by mTOR Modulation.

Cells - 11 Nov 2021

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

Abstract excerpt

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 neuronal lysosomes. Despite the storage phenotype, the role of autophagy and its regulation by mTOR has yet to be explored...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 34831346
DOI: 10.3390/cells10113122
PMCID: PMC8619250

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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