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The galactocerebrosidase enzyme contributes to maintain a functional neurogenic niche during early post-natal CNS development

1 Aug 2012

Abstract excerpt

We report a novel role for the lysosomal galactosylceramidase (GALC), which is defective in globoid cell leukodystrophy (GLD), in maintaining a functional post-natal subventricular zone (SVZ) neurogenic niche. We show that proliferation/self-renewal of neural stem cells (NSCs) and survival of their neuronal and oligodendroglial progeny are impaired in GALC-deficient mice. Using drugs to modulate inflammation and...

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PMID: 22859505
DOI: 10.1093/hmg/dds313

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