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Journal article

Pre-symptomatic development of lower motor neuron connectivity in a mouse model of severe spinal muscular atrophy.

Abstract:

The childhood motor neuron disease spinal muscular atrophy (SMA) results from reduced expression of the survival motor neuron (SMN) gene. Previous studies using in vitro model systems and lower organisms have suggested that low levels of Smn protein disrupt prenatal developmental processes in lower motor neurons, influencing neuronal outgrowth, axon branching and neuromuscular connectivity. The extent to which these developmental pathways contribute to selective vulnerability and pathology in...

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Publication status:
Published

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Publisher copy:
10.1093/hmg/ddp506

Authors


Murray, LM More by this author
Bäumer, D More by this author
Parson, SH More by this author
More by this author
Institution:
University of Oxford
Department:
Oxford, MSD, Clinical Neuroscience
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Journal:
Human molecular genetics
Volume:
19
Issue:
3
Pages:
420-433
Publication date:
2010-02-05
DOI:
EISSN:
1460-2083
ISSN:
0964-6906
URN:
uuid:5d8dac4d-4e84-4b1f-b7d7-e8db09e3ab70
Source identifiers:
133791
Local pid:
pubs:133791

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