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Article

Role of proteolysis in polyglutamine disorders

28 Oct 2003

Abstract excerpt

To date, nine polyglutamine disorders have been characterised, including Huntington's disease (HD), spinobulbar muscular atrophy (SBMA), dentatorubral-pallidoluysian atrophy (DRPLA), and spinocerebellar ataxias 1, 2, 3, 6, 7 and 17 (SCAs). Although knockout and transgenic mouse experiments suggest that a toxic gain of function is central to neuronal death in these diseases (with the probable exception of SCA6),...

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PMID: 14598317
DOI: 10.1002/jnr.10746

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