- 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...
Expand abstract - 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
- Language:
- English
- Keywords:
- Copyright date:
- 2011
Journal article
Reversible molecular pathology of skeletal muscle in spinal muscular atrophy.
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