Oxidative stress is independent of inflammation in the neurodegenerative sanfilippo syndrome type B

Mucopolysaccharidosis (MPS) type IIIB is a genetic deficiency of α‐N‐acetylglucosaminidase, inducing accumulation of partially degraded heparan sulfate (HS) oligosaccharides in tissues. In the central nervous system, this accumulation is associated with microglial activation, neurodegeneration, and...

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Veröffentlicht in:	Journal of neuroscience research 2015-03, Vol.93 (3), p.424-432
Hauptverfasser:	Trudel, Stéphanie, Trécherel, Eric, Gomila, Cathy, Peltier, Maxime, Aubignat, Michaël, Gubler, Brigitte, Morlière, Patrice, Heard, Jean-Michel, Ausseil, Jérôme
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Brain - metabolism Brain - pathology Disease Models, Animal Disease Progression Glutathione Peroxidase - metabolism heparan sulfate inflammation Inflammation - metabolism Inflammation - pathology lysosomal storage diseases Mice Mice, Knockout mucopolysaccharidosis Mucopolysaccharidosis III - metabolism Mucopolysaccharidosis III - pathology NADPH Oxidases - metabolism oxidative stress Oxidative Stress - physiology Superoxide Dismutase - metabolism TLR4 Toll-Like Receptor 4 - genetics
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