ArticleNature communications2020
The Perlman syndrome DIS3L2 exoribonuclease safeguards endoplasmic reticulum-targeted mRNA translation and calcium ion homeostasis.
Article in Nature communications, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers, 1 of them a synthesis that pooled it.
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Who cites it
15 citing papers in PubMed, 1 synthesis or guideline pooled it, 21 citations in OpenAlex.
- Gene expression regulation by CaCellular & molecular biology letters · 2026Pooled it
- The ribonuclease DISL-2 mediates piRNA degradation in C. elegans.Cell reports · 2025Article
- Ribonucleases in Mendelian disease: Characterization and insight from model organisms.Genes & diseases · 2025Review
- Genomic prediction with NetGP based on gene network and multi-omics data in plants.Plant biotechnology journal · 2025Article
- Phenotypic Carney-Stratakis syndrome withProceedings (Baylor University. Medical Center) · 2025Article
- Mesoporous Oxidized Mn-Ca Nanoparticles as Potential Antimicrobial Agents for Wound Healing.Molecules (Basel, Switzerland) · 2024Article
- Biosensors; a novel concept in real-time detection of autophagy.Biosensors & bioelectronics · 2024Review
- Translatomics reveals the role of dietary calcium addition in regulating muscle fat deposition in pigs.Scientific reports · 2024Article
- The SIRT3-ATAD3A axis regulates MAM dynamics and mitochondrial calcium homeostasis in cardiac hypertrophy.International journal of biological sciences · 2024Article
- Conditional ablation of DIS3L2 ribonuclease in pre-meiotic germ cells causes defective spermatogenesis and infertility in male mice.Theranostics · 2024Article
- How hydrolytic exoribonucleases impact human disease: Two sides of the same story.FEBS open bio · 2023Review
- DIS3L2 ribonuclease degrades terminal-uridylated RNA to ensure oocyte maturation and female fertility.Nucleic acids research · 2023Article
- Whole-exome sequencing study identifies four novel gene loci associated with diabetic kidney disease.Human molecular genetics · 2023Article
- A shape-shifting nuclease unravels structured RNA.Nature structural & molecular biology · 2023Article
- The Role of RNA Methylation in Regulating Stem Cell Fate and Function-Focus on mStem cells international · 2021Review
Corrections and comments
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Authors and funding
7 authors at 3 institutions in 1 country.
Funding
Abstract
DIS3L2-mediated decay (DMD) is a surveillance pathway for certain non-coding RNAs (ncRNAs) including ribosomal RNAs (rRNAs), transfer RNAs (tRNAs), small nuclear RNAs (snRNAs), and RMRP. While mutations in DIS3L2 are associated with Perlman syndrome, the biological significance of impaired DMD is obscure and pathological RNAs have not been identified. Here, by ribosome profiling (Ribo-seq) we find specific dysregulation of endoplasmic reticulum (ER)-targeted mRNA translation in DIS3L2-deficient cells. Mechanistically, DMD functions in the quality control of the 7SL ncRNA component of the signal recognition particle (SRP) required for ER-targeted translation. Upon DIS3L2 loss, sustained 3'-end uridylation of aberrant 7SL RNA impacts ER-targeted translation and causes ER calcium leakage. Consequently, elevated intracellular calcium in DIS3L2-deficient cells activates calcium signaling response genes and perturbs ESC differentiation. Thus, DMD is required to safeguard ER-targeted mRNA translation, intracellular calcium homeostasis, and stem cell differentiation.
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Registered trials
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