Elucidating the Biological Role of Neuronal Dystonin Isoforms in the Hereditary Sensory Neuropathy Dystonia Musculorum
Dystonin is a cytoskeletal linker protein whose loss-of-function in dystonia musculorum (dt) mice results in a hereditary sensory neuropathy with profound sensory ataxia. The dystonin gene (Dst) is exceptionally large (~400 kb) producing three giant neuronal dystonin isoforms (>600 kDa) through a...
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| Language: | en |
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Université d'Ottawa / University of Ottawa
2013
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| Online Access: | http://hdl.handle.net/10393/24266 http://dx.doi.org/10.20381/ruor-3063 |