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Modest loss of peripheral axons and formation of brain inclusions in mice with targeted deletion of gigaxonin exon 1

Mutations in gigaxonin are responsible for Giant Axonal Neuropathy (GAN), a progressive neurodegenerative disorder associated with abnormal accumulations of Intermediate Filaments (IFs). Gigaxonin is the substrate-specific adaptor for a new Cul3-E3-ubiquitin ligase family that promotes the proteasom...

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Main Authors: Dequen, Florence, Bomont, Pascale, Gowing, Geneviève, Cleveland, Don W., Julien, Jean-Pierre
格式: Artigo
語言:Inglês
出版: 2008
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在線閱讀:https://ncbi.nlm.nih.gov/pmc/articles/PMC3657508/
https://ncbi.nlm.nih.gov/pubmed/18680552
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1111/j.1471-4159.2008.05601.x
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