Journal article icon

Journal article

The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy.

Abstract:

Spinal muscular atrophy (SMA), which is caused by inactivating mutations in the survival motor neuron 1 (SMN1) gene, is characterized by loss of lower motor neurons in the spinal cord. The gene encoding SMN is very highly conserved in evolution, allowing the disease to be modeled in a range of species. The similarities in anatomy and physiology to the human neuromuscular system, coupled with the ease of genetic manipulation, make the mouse the most suitable model for exploring the basic patho...

Expand abstract

Actions


Access Document


Publisher copy:
10.1242/dmm.007245

Authors


Sleigh, JN More by this author
Gillingwater, TH More by this author
More by this author
Institution:
University of Oxford
Department:
Oxford, MSD, Clinical Neuroscience
Journal:
Disease models and mechanisms
Volume:
4
Issue:
4
Pages:
457-467
Publication date:
2011-07-05
DOI:
EISSN:
1754-8411
ISSN:
1754-8403
URN:
uuid:91f86886-bf04-45c0-bec6-8c544d9e6cda
Source identifiers:
245221
Local pid:
pubs:245221

Terms of use


Metrics



If you are the owner of this record, you can report an update to it here: Report update to this record

TO TOP