Article
Direct evidence for axonal transport defects in a novel mouse model of mutant spastin-induced hereditary spastic paraplegia (HSP) and human HSP patients.
Journal of neurochemistry - 1 Jul 2009
Kasher Paul R, De Vos Kurt J, Wharton Stephen B, Manser Catherine, Bennett Ellen J, Bingley Megan, Wood Jonathan D, Milner Roy, McDermott Christopher J, Miller Christopher C J, Shaw Pamela J, Grierson Andrew J
Abstract excerpt
Mutations in spastin are the most common cause of hereditary spastic paraplegia (HSP) but the mechanisms by which mutant spastin induces disease are not clear. Spastin functions to regulate microtubule organisation, and because of the essential role of microtubules in axonal transport, this has led to the suggestion that defects in axonal transport may underlie at least part of the disease process in HSP....
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