期刊
BIOSCIENCE BIOTECHNOLOGY AND BIOCHEMISTRY
卷 72, 期 9, 页码 2255-2261出版社
TAYLOR & FRANCIS LTD
DOI: 10.1271/bbb.70829
关键词
neurodegeneration; spinobulbar muscular atrophy; androgen receptor; Drosophila; rRNA processing
类别
资金
- Basic Research Activities for Innovative Biosciences (BRAIN)
- Ministry of Education, Culture, Sports, Science, and Technology of Japan
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.
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