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Dystonia-associated mutations cause premature degradation of torsinA protein and cell-type-specific mislocalization to the nuclear envelope

An in-frame 3 bp deletion in the torsinA gene resulting in the loss of a glutamate residue at position 302 or 303 (torsinA ΔE) is the major cause for early-onset torsion dystonia (DYT1). In addition, an 18 bp deletion in the torsinA gene resulting in the loss of residues 323–328 (torsinA Δ323–8) has...

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Detalhes bibliográficos
Main Authors: Giles, Lisa M., Chen, Jue, Li, Lian, Chin, Lih-Shen
Formato: Artigo
Idioma:Inglês
Publicado em: Oxford University Press 2008
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2574948/
https://ncbi.nlm.nih.gov/pubmed/18552369
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddn173
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