Article
Quantitative and functional analyses of spastin in the nervous system: implications for hereditary spastic paraplegia.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 27 Feb 2008
Solowska Joanna M, Morfini Gerardo, Falnikar Aditi, Himes B Timothy, Brady Scott T, Huang Dongyang, Baas Peter W
Abstract excerpt
Spastin and P60-katanin are two distinct microtubule-severing proteins. Autosomal dominant mutations in the SPG4 locus corresponding to spastin are the most common cause of hereditary spastic paraplegia (HSP), a neurodegenerative disease that afflicts the adult corticospinal tracts. Here we sought to evaluate whether SPG4-based HSP is best understood as a "loss-of-function" disease. Using various rat tissues, we...
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