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Abnormal autophagy, ubiquitination, inflammation and apoptosis are dependent upon lysosomal storage and are useful biomarkers of mucopolysaccharidosis VI

BACKGROUND: Lysosomal storage diseases are characterized by intracellular accumulation of metabolites within lysosomes. Recent evidence suggests that lysosomal storage impairs autophagy resulting in accumulation of polyubiquitinated proteins and dysfunctional mitochondria, ultimately leading to apop...

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Detalles Bibliográficos
Autores principales:	Tessitore, Alessandra, Pirozzi, Marinella, Auricchio, Alberto
Formato:	Texto
Lenguaje:	English
Publicado:	BioMed Central 2009
Materias:	Research
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2708151/ https://www.ncbi.nlm.nih.gov/pubmed/19531206 http://dx.doi.org/10.1186/1755-8417-2-4

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