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Reversible molecular pathology of skeletal muscle in spinal muscular atrophy.

Abstract:

Low levels of full-length survival motor neuron (SMN) protein cause the motor neuron disease, spinal muscular atrophy (SMA). Although motor neurons undoubtedly contribute directly to SMA pathogenesis, the role of muscle is less clear. We demonstrate significant disruption to the molecular composition of skeletal muscle in pre-symptomatic severe SMA mice, in the absence of any detectable degenerative changes in lower motor neurons and with a molecular profile distinct from that of denervated m...

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Publisher copy:
10.1093/hmg/ddr360

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Journal:
Human molecular genetics
Volume:
20
Issue:
22
Pages:
4334-4344
Publication date:
2011-11-05
DOI:
EISSN:
1460-2083
ISSN:
0964-6906
URN:
uuid:0b7ae55a-7134-4c7a-a28a-ee7ebaa7744b
Source identifiers:
245226
Local pid:
pubs:245226

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