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Ribosome stalling induced by mutation of a CNS-specific tRNA causes neurodegeneration
Open accessAbstract
Problems making proteins kills nerve cells: Neurodegeneration is associated with a variety of different diseases, but its cellular roots are often obscure. Ishimura et al. find that mutant mice whose brain cells start to die rapidly soon after birth have lost the function of two vital cellular components (see the Perspective by Darnell). The first is a protein that releases stalled ribosomes stuck on messenger RNA (mRNA); the second is a transfer RNA (tRNA), which reads the code for arginine in the mRNA. This tRNA is expressed predominantly in the central nervous system. The lack of the tRNA leads to increased ribosomal stalling at arginine codons, which, when left uncorrected, blocks protein synthesis and proves fatal. Science , this issue p. 455 ; see also p. 378
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“The C57BL6/J (B6J)-nmf205 mouse model has mutations in both the translation GTPase GTPBP2 and the neuronal tRNAArgUCU genes that cause neurodegeneration through ribosomal stalling (Ishimura et al., 2014, 2016).”
From Meta-Analysis of the Alzheimer’s Disease Human Brain Transcriptome and Functional Dissection in Mouse Models (2020) · cites this paper as Ishimura et al., 2014
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Metadata
- Authors
- Ishimura, R., Nagy, G., Dotu, I., Zhou, H., Yang, X.-L., Schimmel, P., Senju, S., Nishimura, Y., Chuang, J. H., & Ackerman, S. L.
- Affiliations
- Howard Hughes Medical Institute (US)
- The Jackson Laboratory for Genomic Medicine, 263 Farmington Avenue, Farmington, CT 06030, USA.
- Scripps Research Institute (US)
- Kumamoto University (JP)
- The Jackson Laboratory for Genomic Medicine (US)
- Corresponding author
- Ishimura, Ryuta
- Nagy, Gabor
- Ackerman, Susan L.
- Venue
- Science
- Published
- 2014-07-25
- Volume
- 345
- Issue
- 6195
- Pages
- 455-459
- ISSN
- 0036-8075 (ISSN-L)
- 1095-9203
- PMC
- PMC4281038
- OpenAlex
- W1983859619
- Cited by
- 455
- References
- 22
- Citations by year
- 2026: 29 · 2025: 34 · 2024: 41 · 2023: 47 · 2022: 33 · 2021: 40 · 2020: 44 · 2019: 51 · 2018: 42 · 2017: 30 · 2016: 30 · 2015: 24 · 2014: 9
- FWCI
- 12.72
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Science (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- The Mouseion at the JAXlibrary (Jackson Laboratory) (submittedVersion)
- Funders
- National Science Foundation
- Howard Hughes Medical Institute
- Natural Environment Research Council
- National Cancer Institute (#CA34196, P30CA034196)
- National Institute of Neurological Disorders and Stroke (R01NS085092)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › RNA and protein synthesis mechanisms
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › RNA modifications and cancer
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Keywords
- ribosome stalling, tRNA mutations, pelota, ribosome recycling, neurodegeneration
- MeSH terms
- Animals, Cell Nucleus, Cerebellum, Endonucleases, Mice, Inbred BALB C, Mice, Inbred C57BL, Microfilament Proteins, Ribosomes, RNA, Transfer, Arg, Protein Biosynthesis, Point Mutation, Cell Cycle Proteins, GTP-Binding Proteins, Neurodegenerative Diseases, RNA Splice Sites, Mice
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