RNA-Binding Protein Hoip Accelerates PolyQ-Induced Neurodegeneration in Drosophila
Open accessAbstract
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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Other genetic screens of polyQ toxicity have uncovered RNA-binding proteins, but those genes do not overlap with the modifiers presented in this study.
“Other genetic screens of polyQ toxicity have also uncovered RNA-binding proteins (77–79), although those genes identified do not overlap with the modifiers presented in this study.”
From Functional genomic screen and network analysis reveal novel modifiers of tauopathy dissociated from tau phosphorylation (2011) · cites this paper as [79]
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Ambegaokar, S. S., & Jackson, G. R. (2011) · Human Molecular Genetics · doi:10.1093/hmg/ddr432 · Cited by 113 · see citing works
- Authors
- MURATA, T., SUZUKI, E., ITO, S., SAWATSUBASHI, S., ZHAO, Y., YAMAGATA, K., TANABE, M., FUJIYAMA, S., KIMURA, S., UEDA, T., MATSUKAWA, H., KOUZMENKO, A., FURUTANI, T., KURANAGA, E., MIURA, M., TAKEYAMA, K.-I., & KATO, S.
- Affiliations
- Institute for Quantitative Biosciences, The University of Tokyo (JP)
- Japan Science and Technology Agency (JP)
- Astellas Pharma (Japan) (JP)
- The University of Tokyo (JP)
- ORCID
- Venue
- Bioscience, Biotechnology, and Biochemistry
- Publisher
- Oxford University Press (OUP)
- Published
- 2008-09-23
- Volume
- 72
- Issue
- 9
- Pages
- 2255-2261
- ISSN
- 0916-8451 (ISSN-L)
- 1347-6947
- OpenAlex
- W1991201531
- Cited by
- 15
- References
- 3
- Citations by year
- 2026: 1 · 2025: 1 · 2023: 1 · 2021: 1 · 2020: 1 · 2016: 1 · 2014: 2 · 2013: 2
- FWCI
- 0.72
- Type
- journal article
- Language
- en
- Open access
- Licence
- cc-by-nc-nd
- Locations
- Bioscience Biotechnology and Biochemistry (publishedVersion)
- PubMed (publishedVersion)
- Institutional Repositories DataBase (IRDB) (submittedVersion, cc-by-nc-nd)
- Funders
- Center for Unmanned Aircraft Systems
- Topics
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Keywords
- HOIP, polyglutamine expansion, spinal and bulbar muscular atrophy, androgen receptor, neurodegeneration, 18S rRNA, NOP56, rRNA processing, RNA-binding proteins, Drosophila model
- MeSH terms
- Animals, Cells, Cultured, Disease Models, Animal, Drosophila, Eye, Mutation, Nerve Degeneration, Peptides, Receptors, Androgen, Transfection, RNA-Binding Proteins, Genes, Insect, Muscular Disorders, Atrophic, Drosophila Proteins, Animals, Genetically Modified
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