RNA-binding protein hoip accelerates polyQ-induced neurodegeneration in Drosophila

Takuya Murata, Eriko Suzuki, Saya Ito, Shun Sawatsubashi, Yue Zhao, Kaoru Yamagata, Masahiko Tanabe, Sally Fujiyama, Shuhei Kimura, Takashi Ueda, Hiroyuki Matsukawa, Alexander Kouzmenko, Takashi Furutani, Erina Kuranaga, Masayuki Miura, Ken Ichi Takeyama, Shigeaki Kato

Research output: Contribution to journalArticlepeer-review

11 Citations (Scopus)


Abnormal polyglutamine (polyQ) expansion in the N-terminal domain of the human androgen receptor (hAR) is known to cause spinobulbar muscular atrophy (SBMA), a hereditary human neurodegenerative disorder. To explore the molecular mechanisms of neurodegeneration in SBMA, we genetically screened modulators of neurodegeneration in a Drosophila SBMA experimental model system. We identified hoip as an accelerator of polyQ-induced neurodegeneration. We found that hoip forms a complex with 18s rRNA together nop56 and nop5 proteins, whose human homologs are known to form a snoRNP complex involved in ribosomal RNA processing. Significantly, the levels of mutant polyQ-hAR were up-regulated in a mutant line overexpressing hoip. Consistently, severe neurodegeneration phenotype (rough eye) was also observed in both nop56 and nop5 overexpression mutant lines. These findings suggest that the process of neurodegeneration induced by abnormal polyQ expansion in the hAR may be regulated by the activity of snoRNP complex.

Original languageEnglish
Pages (from-to)2255-2261
Number of pages7
JournalBioscience, Biotechnology and Biochemistry
Issue number9
Publication statusPublished - 2008
Externally publishedYes


  • Androgen receptor
  • Drosophila
  • Neurodegeneration
  • Spinobulbar muscular atrophy
  • rRNA processing

ASJC Scopus subject areas

  • Biotechnology
  • Analytical Chemistry
  • Biochemistry
  • Applied Microbiology and Biotechnology
  • Molecular Biology
  • Organic Chemistry


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