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Journal article

SMN deficiency disrupts brain development in a mouse model of severe spinal muscular atrophy.

Abstract:

Reduced expression of the survival motor neuron (SMN) gene causes the childhood motor neuron disease spinal muscular atrophy (SMA). Low levels of ubiquitously expressed SMN protein result in the degeneration of lower motor neurons, but it remains unclear whether other regions of the nervous system are also affected. Here we show that reduced levels of SMN lead to impaired perinatal brain development in a mouse model of severe SMA. Regionally selective changes in brain morphology were apparent...

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Publication status:
Published

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Publisher copy:
10.1093/hmg/ddq340

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Journal:
Human molecular genetics
Volume:
19
Issue:
21
Pages:
4216-4228
Publication date:
2010-11-05
DOI:
EISSN:
1460-2083
ISSN:
0964-6906
URN:
uuid:d6ab5f33-5238-40d1-99c1-251583127cb1
Source identifiers:
241514
Local pid:
pubs:241514

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