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Article

Cleavage, aggregation and toxicity of the expanded androgen receptor in spinal and bulbar muscular atrophy

1 Apr 1998

Abstract excerpt

Spinal and bulbar muscular atrophy (SBMA) is a neurodegenerative disease caused by the expansion of a polyglutamine repeat within the androgen receptor (AR). We have studied the mutant AR in an in vitro system, and find both aggregation and proteolytic processing of the AR protein to occur in a polyglutamine repeat length-dependent manner. In addition, we find the aberrant metabolism of expanded repeat AR to be...

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PMID: 9499423
DOI: 10.1093/hmg/7.4.693

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