The spinocerebellar ataxia type 1 protein, ataxin-1, has RNA-binding activity that is inversely affected by the length of its polyglutamine tract
Abstract
Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant neurodegenerative disease caused by the expansion of a polyglutamine tract within the SCA1 product, ataxin-1. Previously, using transgenic mice, it was demonstrated that in order for a mutant allele of ataxin-1 to cause disease it must be transported to the nucleus of the neuron. Using an in vitro RNA-binding assay, we demonstrate that ataxin-1 does bind RNA and that this binding diminishes as the length of its polyglutamine tract increases. These observations suggest that ataxin-1 plays a role in RNA metabolism and that the expansion of the polyglutamine tract may alter this function.
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RNA regulation may play a significant role in polyQ toxicity.
“RNA regulation may also play a significant role in polyQ toxicity as well, as the CAG repeats in polyglutamine-encoding RNAs can induce toxicity (74,75), and polyglutamine proteins can also bind RNA with an affinity dependent on the polyQ expansion length (76).”
From Functional genomic screen and network analysis reveal novel modifiers of tauopathy dissociated from tau phosphorylation (2011) · cites this paper as [76]
Polyglutamine proteins can bind RNA with an affinity dependent on the polyQ expansion length.
“and polyglutamine proteins can also bind RNA with an affinity dependent on the polyQ expansion length (76).”
From Functional genomic screen and network analysis reveal novel modifiers of tauopathy dissociated from tau phosphorylation (2011) · cites this paper as [76]
Cites
Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1
Orr, H. T., Chung, M.-Y., Banfi, S., Kwiatkowski, T. J., Servadio, A., Beaudet, A. L., McCall, A. E., Duvick, L. A., Ranum, L. P. W., & Zoghbi, H. Y. (1993) · Nature Genetics · doi:10.1038/ng0793-221 · Cited by 1,632 · cited 2× · see citing works
“Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant neurodegenerative disease caused by the expansion of a polyglutamine tract within the SCA1 product, ataxin-1 (1).”
BackgroundSelf-citationScherzinger, E., Lurz, R., Turmaine, M., Mangiarini, L., Hollenbach, B., Hasenbank, R., Bates, G. P., Davies, S. W., Lehrach, H., & Wanker, E. E. (1997) · Cell · doi:10.1016/s0092-8674(00)80514-0 · Cited by 1,291 · see citing works
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circRNA biogenesis competes with pre-mRNA splicing
Ashwal-Fluss, R., Meyer, M., Pamudurti, N. R., Ivanov, A., Bartok, O., Hanan, M., Evantal, N., Memczak, S., Rajewsky, N., & Kadener, S. (2014) · Molecular Cell · doi:10.1016/j.molcel.2014.08.019 · Cited by 3,083 · see citing works
CLIP Identifies Nova-Regulated RNA Networks in the Brain
Ule, J., Jensen, K. B., Ruggiu, M., Mele, A., Ule, A., & Darnell, R. B. (2003) · Science · doi:10.1126/science.1090095 · Cited by 1,120 · see citing works
Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1
Lim, J., Crespo-Barreto, J., Jafar-Nejad, P., Bowman, A. B., Richman, R., Hill, D. E., Orr, H. T., & Zoghbi, H. Y. (2008) · Nature · doi:10.1038/nature06731 · Cited by 311 · see citing works
Ambegaokar, S. S., & Jackson, G. R. (2011) · Human Molecular Genetics · doi:10.1093/hmg/ddr432 · Cited by 113 · see citing works
“RNA regulation may also play a significant role in polyQ toxicity as well, as the CAG repeats in polyglutamine-encoding RNAs can induce toxicity (74,75), and polyglutamine proteins can also bind RNA with an affinity dependent on the polyQ expansion length (76).”
Background
- Authors
- Yue, S.
- Affiliations
- University of Minnesota (US)
- Corresponding author
- Yue, S.
- Venue
- Human Molecular Genetics
- Publisher
- Oxford University Press (OUP)
- Published
- 2001-01-01
- Volume
- 10
- Issue
- 1
- Pages
- 25-30
- ISSN
- 0964-6906 (ISSN-L)
- 1460-2083
- OpenAlex
- W2159178453
- Cited by
- 168
- References
- 2
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 5 · 2023: 8 · 2022: 2 · 2021: 7 · 2020: 9 · 2019: 5 · 2018: 5 · 2017: 3 · 2016: 5 · 2015: 3 · 2014: 7 · 2013: 8 · 2012: 8
- FWCI
- 3.29
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Human Molecular Genetics (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (NS22920)
- National Institute of Neurological Disorders and Stroke (NS-22920)
- 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
- spinocerebellar ataxia type 1, ataxin-1, RNA-binding activity, RNA metabolism, neurodegeneration, nuclear transport, transgenic mice
- MeSH terms
- Ataxins, Ataxin-1, Dose-Response Relationship, Drug, Humans, Nerve Tissue Proteins, Nuclear Proteins, Peptides, Plasmids, Protein Binding, RNA, Transcription, Genetic, Protein Biosynthesis, DNA, Complementary, Spinocerebellar Ataxias
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