ArticleThe Journal of clinical investigation2018
Ribonuclease inhibitor 1 regulates erythropoiesis by controlling GATA1 translation.
Article in The Journal of clinical investigation, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 26 citations in OpenAlex.
- Ocular Manifestations of Infectious Salmon Anaemia Virus Infection in Atlantic Salmon (Salmo salar).Journal of fish diseases · 2026Article
- Cytoplasmic PXR regulates glucose metabolism by binding mRNAs and modulating their stability.Nature structural & molecular biology · 2025Article
- Epitope-focused vaccine immunogens design using tailored horseshoe-shaped scaffold.Journal of nanobiotechnology · 2025Article
- Ribonuclease inhibitor and angiogenin system regulates cell type-specific global translation.Science advances · 2024Article
- Disruption of mitochondrial energy metabolism is a putative pathogenesis of Diamond-Blackfan anemia.iScience · 2024Article
- Critical Differential Expression Assessment for Individual Bulk RNA-Seq Projects.bioRxiv : the preprint server for biology · 2024Article
- Ribonuclease inhibitor 1 (RNH1) deficiency cause congenital cataracts and global developmental delay with infection-induced psychomotor regression and anemia.European journal of human genetics : EJHG · 2023Article
- Human molecular genetics sheds light on the physiological significance of ribonuclease inhibitor (RNH1).European journal of human genetics : EJHG · 2023Article
- Exploring the mechanistic link between SF3B1 mutation and ring sideroblast formation in myelodysplastic syndrome.Scientific reports · 2022Article
- LRR-protein RNH1 dampens the inflammasome activation and is associated with COVID-19 severity.Life science alliance · 2022Article
- Research progress on the tsRNA classification, function, and application in gynecological malignant tumors.Cell death discovery · 2021Review
- Translational control of stem cell function.Nature reviews. Molecular cell biology · 2021Review
- Angiogenin (ANG)-Ribonuclease Inhibitor (RNH1) System in Protein Synthesis and Disease.International journal of molecular sciences · 2021Review
- Diamond-Blackfan anemia.Blood · 2020Review
- GATA1 mutations in red cell disorders.IUBMB life · 2020Review
- An update on the pathogenesis and diagnosis of Diamond-Blackfan anemia.F1000Research · 2018Review
- Regulation of IRE1 RNase activity by the Ribonuclease inhibitor 1 (RNH1).Cell cycle (Georgetown, Tex.) · 2018Article
Corrections and comments
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Authors and funding
18 authors at 8 institutions in 4 countries.
Funding
Abstract
Ribosomal proteins (RP) regulate specific gene expression by selectively translating subsets of mRNAs. Indeed, in Diamond-Blackfan anemia and 5q- syndrome, mutations in RP genes lead to a specific defect in erythroid gene translation and cause anemia. Little is known about the molecular mechanisms of selective mRNA translation and involvement of ribosomal-associated factors in this process. Ribonuclease inhibitor 1 (RNH1) is a ubiquitously expressed protein that binds to and inhibits pancreatic-type ribonucleases. Here, we report that RNH1 binds to ribosomes and regulates erythropoiesis by controlling translation of the erythroid transcription factor GATA1. Rnh1-deficient mice die between embryonic days E8.5 and E10 due to impaired production of mature erythroid cells from progenitor cells. In Rnh1-deficient embryos, mRNA levels of Gata1 are normal, but GATA1 protein levels are decreased. At the molecular level, we found that RNH1 binds to the 40S subunit of ribosomes and facilitates polysome formation on Gata1 mRNA to confer transcript-specific translation. Further, RNH1 knockdown in human CD34+ progenitor cells decreased erythroid differentiation without affecting myelopoiesis. Our results reveal an unsuspected role for RNH1 in the control of GATA1 mRNA translation and erythropoiesis.
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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.