Publications

Dernière publication 21/10/2009

Grafting Neural Precursor Cells Promotes Functional Recovery in an SCA1 Mouse Mo

The B05 transgenic SCA1 mice, expressing human ataxin-1 with an expanded polyglutamine tract in cerebellar Purkinje cells (PCs), recapitulate many...

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    [title] => Grafting Neural Precursor Cells Promotes Functional Recovery in an SCA1 Mouse Mo
    [paragraph] => Grafting Neural Precursor Cells Promotes Functional Recovery in an SCA1 Mouse Model.
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Authors
S. Chintawar, R. Hourez, A. Ravella, D. Gall, D. Orduz et al.

 Lab
Brussels Free University (ULB), Laboratory of Experimental Neurology and Laboratory of Neurophysiology, Brussels, Belgium ; University of Turin, Department of Clinical and Biological Sciences and Cavalieri Ottolenghi Scientific Institute, Orbassano, Italy
Journal
The Journal of Neuroscience
Abstract
The B05 transgenic SCA1 mice, expressing human ataxin-1 with an expanded polyglutamine tract in cerebellar Purkinje cells (PCs), recapitulate many pathological and behavioral characteristics of the neurodegenerative disease spinocerebellar ataxia type 1 (SCA1), including progressive ataxia and PC loss. We transplanted neural precursor cells (NPCs) derived from the subventricular zone of GFP-expressing adult mice into the cerebellar white matter of SCA1 mice when they showed absent (5 weeks), initial (13 weeks), and significant (24 weeks) PC loss. Only in mice with significant cell loss, grafted NPCs migrated into the cerebellar cortex. These animals showed improved motor skills compared with sham-treated controls. No grafted cell adopted the morphological and immunohistochemical characteristics of PCs, but the cerebellar cortex in NPC-grafted SCA1 mice had a significantly thicker molecular layer and more surviving PCs. Perforated patch-clamp recordings revealed a normalization of the PC basal membrane potential, which was abnormally depolarized in sham-treated animals. No significant increase in levels of several neurotrophic factors was observed, suggesting, along with morphological observation, that the neuroprotective effect of grafted NPCs was mediated by direct contact with the host PCs. We postulate that a similar neuroprotective effect of NPCs may be applicable to other cerebellar degenerative diseases.
BIOSEB Instruments Used
Grip strength test (BIO-GS3)

Keywords/Topics
Domaines de recherche divers
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Une méthode simple pour quantifier objectivement la force musculaire des rats et souris et l'effet de drogues, toxines, maladies musculaires (ex: myopathie) et neurodégénératives. Cette mesure de force est souvent employée en association avec le test de coordination motrice ROTAROD: un sujet présentant une coordination normale montrera des résultats médiocres en cas de faible force musculaire. Un must pour vos recherches sur l'activité, la coordination et le contrôle musculaire: particulièrement utile pour vos études sur les maladies de Parkinson et Huntington.
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