ArticleNature2013
CLP1 links tRNA metabolism to progressive motor-neuron loss.
Article in Nature, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 170 papers.
What it found
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Who cites it
170 citing papers in PubMed, 262 citations in OpenAlex.
- Ashwin and FAM98 paralogs define nuclear and cytoplasmic RNA ligase complexes for tRNA biogenesis.Nature communications · 2026Article
- Enzymology of the metazoan tRNA ligase complex: a lifetime in cycles.Cellular and molecular life sciences : CMLS · 2026Review
- EXOSC3 G191 Variants Trigger System-Wide Recalibration of RNA Processing Machinery.bioRxiv : the preprint server for biology · 2026Article
- A Rare Case of PCH10 With CLP1 Mutation: Clinical Features and Dental Management of Riga-Fede Lesion in a Pediatric Patient.Case reports in dentistry · 2026Article
- Unlocking the role of tRNA-derived fragments in shaping breast cancer signaling.Naunyn-Schmiedeberg's archives of pharmacology · 2025Review
- tRNA-derived small noncoding RNAs: Roles in brain aging and neurodegenerative disorders.Zoological research · 2025Review
- Circadian Regulation of Neuronal Membrane Capacitance-Mechanisms and Implications for Neural Computation and Behavior.International journal of molecular sciences · 2025Review
- tsRNA modifications: An emerging layer of biological regulation in disease.Journal of advanced research · 2025Review
- Emerging roles of transfer RNA fragments in the CNS.Brain : a journal of neurology · 2025Review
- tsRNA, cell death and disease: Connecting the dots.Non-coding RNA research · 2025Review
- Queuosine is incorporated into precursor tRNA before splicing.Nature communications · 2025Article
- Polyglycine-mediated aggregation of FAM98B disrupts tRNA processing in GGC repeat disorders.Science (New York, N.Y.) · 2025Article
- Gammaherpesvirus infection triggers the formation of tRNA fragments from premature tRNAs.mBio · 2025Article
- Exploring the Potential of tsRNA as Biomarkers for Diagnosis and Treatment of Neurogenetic Disorders.Molecular neurobiology · 2025Review
- Mechanistic basis for PYROXD1-mediated protection of the human tRNA ligase complex against oxidative inactivation.Nature structural & molecular biology · 2025Article
- tRF-5028c disrupts trophoblast function in recurrent spontaneous abortion by inhibiting CRKL-mediated Rap1 signaling pathway.Cellular & molecular biology letters · 2025Article
- Deciphering the role of tRNA-derived fragments in neurological and psychiatric disease pathogenesis.Frontiers in cellular neuroscience · 2025Review
- tsRNA-5006c regulates hippocampal neurons ferroptosis to ameliorate perioperative neurocognitive disorders in aged male mice.3 Biotech · 2025Article
- Cabbage Exosome-Like Nanoparticles Encapsulating Small Noncoding tsRNA Prevent Postinjury Arterial Restenosis.Research (Washington, D.C.) · 2025Article
- Review
110 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
24 authors at 15 institutions in 5 countries.
Funding
Abstract
CLP1 was the first mammalian RNA kinase to be identified. However, determining its in vivo function has been elusive. Here we generated kinase-dead Clp1 (Clp1(K/K)) mice that show a progressive loss of spinal motor neurons associated with axonal degeneration in the peripheral nerves and denervation of neuromuscular junctions, resulting in impaired motor function, muscle weakness, paralysis and fatal respiratory failure. Transgenic rescue experiments show that CLP1 functions in motor neurons. Mechanistically, loss of CLP1 activity results in accumulation of a novel set of small RNA fragments, derived from aberrant processing of tyrosine pre-transfer RNA. These tRNA fragments sensitize cells to oxidative-stress-induced p53 (also known as TRP53) activation and p53-dependent cell death. Genetic inactivation of p53 rescues Clp1(K/K) mice from the motor neuron loss, muscle denervation and respiratory failure. Our experiments uncover a mechanistic link between tRNA processing, formation of a new RNA species and progressive loss of lower motor neurons regulated by p53.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.