Journal article
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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Authors
Bibliographic Details
- Journal:
- Human molecular genetics
- Volume:
- 20
- Issue:
- 22
- Pages:
- 4334-4344
- Publication date:
- 2011-11-01
- DOI:
- EISSN:
-
1460-2083
- ISSN:
-
0964-6906
- Source identifiers:
-
245226
Item Description
- Language:
- English
- Keywords:
- Pubs id:
-
pubs:245226
- UUID:
-
uuid:0b7ae55a-7134-4c7a-a28a-ee7ebaa7744b
- Local pid:
- pubs:245226
- Deposit date:
- 2012-12-19
Terms of use
- Copyright date:
- 2011
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