Astrocytes and lysosomal storage diseases

Highlights • Several lysosomal storage diseases (LSDs) are associated with CNS neuron loss. • Astrocyte dysfunction is observed in numerous LSDs and is suggested to impact neurodegeneration. • Enzyme/gene replacement in astrocytes reduces neuron loss, implicating astrocyte dysfunction in LSD patholo...

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Veröffentlicht in:	Neuroscience 2016-05, Vol.323, p.195-206
Hauptverfasser:	Rama Rao, K.V, Kielian, T
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals astrocytes Astrocytes - physiology Humans lysosomal storage diseases Lysosomal Storage Diseases - genetics Lysosomal Storage Diseases - physiopathology mitochondrial dysfunction neurodegeneration Neurodegenerative Diseases - genetics Neurodegenerative Diseases - physiopathology Neurology Neurons - physiology reactive astrocytosis
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Zusammenfassung:	Highlights • Several lysosomal storage diseases (LSDs) are associated with CNS neuron loss. • Astrocyte dysfunction is observed in numerous LSDs and is suggested to impact neurodegeneration. • Enzyme/gene replacement in astrocytes reduces neuron loss, implicating astrocyte dysfunction in LSD pathology.
ISSN:	0306-4522 1873-7544
DOI:	10.1016/j.neuroscience.2015.05.061