Oligodendrocyte development and CNS myelination are unaffected in a mouse model of severe spinal muscular atrophy.

Autor:	O'Meara RW; Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada.; Department of Cellular and Molecular Medicine, University of Ottawa, Ottawa, ON, Canada.; University of Ottawa Centre for Neuromuscular Disease, Ottawa, Ontario, Canada., Cummings SE; Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada.; Department of Cellular and Molecular Medicine, University of Ottawa, Ottawa, ON, Canada.; University of Ottawa Centre for Neuromuscular Disease, Ottawa, Ontario, Canada., De Repentigny Y; Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada.; University of Ottawa Centre for Neuromuscular Disease, Ottawa, Ontario, Canada., McFall E; Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada.; University of Ottawa Centre for Neuromuscular Disease, Ottawa, Ontario, Canada., Michalski JP; Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada.; Department of Cellular and Molecular Medicine, University of Ottawa, Ottawa, ON, Canada.; University of Ottawa Centre for Neuromuscular Disease, Ottawa, Ontario, Canada., Deguise MO; Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada.; Department of Cellular and Molecular Medicine, University of Ottawa, Ottawa, ON, Canada.; University of Ottawa Centre for Neuromuscular Disease, Ottawa, Ontario, Canada., Gibeault S; Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada.; University of Ottawa Centre for Neuromuscular Disease, Ottawa, Ontario, Canada., Kothary R; Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada.; Department of Cellular and Molecular Medicine, University of Ottawa, Ottawa, ON, Canada.; University of Ottawa Centre for Neuromuscular Disease, Ottawa, Ontario, Canada.; Department of Medicine, University of Ottawa, Ottawa, ON, Canada.
Jazyk:	angličtina
Zdroj:	Human molecular genetics [Hum Mol Genet] 2017 Jan 15; Vol. 26 (2), pp. 282-292.
DOI:	10.1093/hmg/ddw385
Abstrakt:	The childhood neurodegenerative disease spinal muscular atrophy (SMA) is caused by loss-of-function mutations or deletions in the Survival Motor Neuron 1 (SMN1) gene resulting in insufficient levels of survival motor neuron (SMN) protein. Classically considered a motor neuron disease, increasing evidence now supports SMA as a multi-system disorder with phenotypes discovered in cortical neuron, astrocyte, and Schwann cell function within the nervous system. In this study, we sought to determine whether Smn was critical for oligodendrocyte (OL) development and central nervous system myelination. A mouse model of severe SMA was used to assess OL growth, migration, differentiation and myelination. All aspects of OL development and function studied were unaffected by Smn depletion. The tremendous impact of Smn depletion on a wide variety of other cell types renders the OL response unique. Further investigation of the OLs derived from SMA models may reveal disease modifiers or a compensatory mechanism allowing these cells to flourish despite the reduced levels of this multifunctional protein. (© The Author 2016. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)
Databáze:	MEDLINE
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