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The therapeutic potential of neural stem/progenitor cells in murine globoid cell leukodystrophy is conditioned by macrophage/microglia activation

Authors :
Serena Pellegatta
Patrizia Tunici
Pietro Luigi Poliani
Diego Dolcetta
Laura Cajola
Cristina Colombelli
Emilio Ciusani
Stefano Di Donato
Gaetano Finocchiaro
Source :
Neurobiology of Disease, Vol 21, Iss 2, Pp 314-323 (2006)
Publication Year :
2006
Publisher :
Elsevier, 2006.

Abstract

Twitcher (GALCtwi/twi) is the murine model of globoid cell leukodystrophy (GLD or Krabbe disease), a disease caused by mutations of the lysosomal enzyme galactocerebrosidase (GALC). To verify the therapeutic potential on twitcher of neural stem/progenitor cells (NSPC), we transduced them with a GALC lentiviral vector. Brain injection of NSPC-GALC increased survival of GALCtwi/twi from 36.1 ± 4.1 to 52.2 ± 5.6 days (P

Details

Language :
English
ISSN :
1095953X
Volume :
21
Issue :
2
Database :
Directory of Open Access Journals
Journal :
Neurobiology of Disease
Publication Type :
Academic Journal
Accession number :
edsdoj.4325cc85d9894a2396c95274ade04de8
Document Type :
article
Full Text :
https://doi.org/10.1016/j.nbd.2005.07.016