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

@article{Merry1998CleavageAA,
  title={Cleavage, aggregation and toxicity of the expanded androgen receptor in spinal and bulbar muscular atrophy.},
  author={Diane E. Merry and Yasushi Kobayashi and Christine K Bailey and Addis A Taye and Kenneth H. Fischbeck},
  journal={Human molecular genetics},
  year={1998},
  volume={7 4},
  pages={693-701}
}
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 coupled to cellular toxicity, indicating a likely molecular basis for the toxic gain… CONTINUE READING

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Genetics Instabilities and Hereditary Neurological Diseases

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