bims-spribo Biomed News
on Specialized ribosomes
Issue of 2026–08–30
sixteen papers selected by
Maxim Bouvet, Università di Torino
  1. Ribosome heterogeneity provides a translational barrier against protein misfolding disease in Saccharomyces cerevisiae.
  2. The Role of ZNF598 in Translational Quality Control: Mechanisms and Emerging Biological Functions.
  3. The N-terminal Extension of Ribosomal Protein Rps2 Shapes Its Early Fate through Chaperone-Importin Competition and Arginine Methylation.
  4. MetaTIS: a tool to predict cognate and near-cognate translation initiation sites in human.
  5. hY3 RNA binds and inhibits ribosomes and modulates the starvation-induced integrated stress response.
  6. 16S ribosomal RNA modification drives transcript-specific translation efficiency.
  7. Tcf15 promotes an open nucleolar chromatin state to safeguard ribosome biogenesis and genome stability in mouse embryonic stem cells.
  8. RNA modifications and RNA-binding proteins: interactions, regulation, and new avenues for therapeutic modulation.
  9. A Luciferase-Based Assay for Assessing Cap-Independent Translation in Wheat Germ Extract.
  10. Ribosomal proteins, miRNAs, and extracellular vesicles in myelodysplastic syndromes: molecular regulation and biomarker potential.
  11. Remaking an exit: dynamic regulation of ER exit sites by post-translational modifications.
  12. RNA aptamers for sodium and lithium are abundant in mammals.
  13. The proportional scaling of mRNA and ribosome concentrations controls eukaryotic cell growth.
  14. RPLP0 identified as a ferroptosis regulator in HCC through pan-cancer analysis.
  15. Mitochondrial ribosome content as a proxy for respiration.
  16. Muscular dystrophy-associated lamin variants disrupt cellular organization through a nucleolar-ribosomal axis.
  1. Genetics. 2026 Aug 22. pii: iyag220. [Epub ahead of print]
    Ribosome heterogeneity provides a translational barrier against protein misfolding disease in Saccharomyces cerevisiae.
    Min Suk Jang, Sia Han, Eunsil Choi, Jihwan Hwang, Moonil Son.
      The yeast prion [PSI+] is a self-propagating amyloid of the translation termination factor Sup35p (eRF3). Among 79 ribosomal proteins in Saccharomyces cerevisiae, through a genetic screen for novel anti-prion factors, we identified RPL24B, a 60S ribosomal subunit protein, as a key determinant of prion generation. Our findings revealed that the functional distinction between the ribosomal paralogs RPL24A and RPL24B is the primary driver of ribosomal heterogeneity-based anti-prion generation. Heterogeneity establishes a critical genetic checkpoint that strictly regulates the emergence and heritable diversity of prions and their variants. We demonstrate that deleting RPL24B (rpl24bΔ) significantly increased the frequency of [PSI+] generation compared to the wild-type strain. Crucially, the resulting [PSI+] prions in the rpl24bΔ mutants exhibited distinct heritable diversity, characterized by high thermostability and exceptionally high propagon numbers. This anti-prion activity is uniquely specific to the RPL24B paralog, because deletion of its highly homologous counterpart, RPL24A, does not reproduce the effects observed in rpl24bΔ. Mechanistically, we identified valine 138 (V138) in Rpl24b as the major molecular determinant of its anti-prion generation activity. Loss of Rpl24b perturbs the cellular protein quality control landscape, leading to high accumulation of misfolded protein aggregates. This work suggests that RPL24B functions as a specialized retainer of genetic robustness, providing a translational barrier against protein misfolding diseases by filtering out robust amyloid variants.
    Keywords:  Ribosomal protein; [PSI+]; [URE3]; prion; yeast
    DOI:  https://doi.org/10.1093/genetics/iyag220
  2. Biology (Basel). 2026 Aug 20. pii: 1435. [Epub ahead of print]15(16):
    The Role of ZNF598 in Translational Quality Control: Mechanisms and Emerging Biological Functions.
    Siyuan Wu, Zhiqian Liu, Guodong Chen.
      During protein synthesis, ribosome stalling, collision, and aberrant elongation can lead to the accumulation of defective nascent polypeptides and compromise cellular homeostasis. To counteract such translational disturbances, eukaryotic cells have evolved a highly conserved translational quality control network, in which the ribosome-associated quality control (RQC) pathway plays a central role in the recognition and elimination of aberrant translation complexes. Zinc Finger Protein 598 (ZNF598), a key E3 ubiquitin ligase in mammalian cells, functions as an essential factor in the early recognition and signal transduction steps of the RQC pathway. Accumulating evidence indicates that ZNF598 senses aberrant translational states, and particularly in the context of ribosome collision, mediates site-specific ubiquitination of 40S ribosomal proteins, thereby promoting ribosome splitting, nascent chain clearance, and subsequent processing of defective mRNAs. Beyond its canonical role in RQC, ZNF598 has also been implicated in the translational repression of defective mRNAs, regulation of inflammatory signaling, antiviral responses, and control of toxic translation products associated with neurodegenerative disorders. In this review, we summarize the structural features, molecular mechanisms, regulatory networks, and physiological as well as pathological functions of ZNF598. We also discuss current controversies and future directions in the field, with the aim of providing a broader framework for understanding translational quality control and its therapeutic potential.
    Keywords:  GIGYF2; ZNF598; ribosome collision; ribosome-associated quality control; translational stalling; ubiquitination
    DOI:  https://doi.org/10.3390/biology15161435
  3. J Biol Chem. 2026 Aug 25. pii: S0021-9258(26)02353-7. [Epub ahead of print] 113481
    The N-terminal Extension of Ribosomal Protein Rps2 Shapes Its Early Fate through Chaperone-Importin Competition and Arginine Methylation.
    Maximilian Mack, Benjamin Pillet, Nicole Holton, Ingrid Zierler, Stephanie Himpich, Phyllis Jessen, Markus C Wahl, Dieter Kressler, Brigitte Pertschy.
      Efficient nuclear import of ribosomal proteins is essential for the timely assembly of ribosomal subunit precursors in the nucleus and progression of ribosome biogenesis. Several ribosomal proteins are escorted to the nucleus by dedicated chaperones, which shield their interaction surfaces and assist their delivery. Here, we characterize the N-terminal extension of the small subunit ribosomal protein Rps2 as a regulatory hub that integrates binding of its dedicated chaperone Tsr4, recognition by its importin Pse1, and arginine methylation. By mapping Pse1's interaction interface on Rps2's N-terminal extension, we identified arginine 11 (R11), a known methylation site, as a critical residue. We demonstrate that Rps2 is already methylated while associated with Tsr4, indicating that methylation occurs at an early stage of the Rps2 assembly path prior to nuclear import. We further show that the chaperone Tsr4 and the importin Pse1 compete for binding to the Rps2 N-terminal extension. While Tsr4 binds this region with higher affinity than Pse1, Pse1 also contacts additional regions within full-length Rps2. Arginine methylation of Rps2 modestly reduces Pse1 binding to the Rps2 N-terminal extension, while having little effect on Tsr4 binding, suggesting that this modification can influence importin recognition without disrupting chaperone association. Finally, we find that Rps2 methylation increases at low temperature and that loss of Hmt1-mediated methylation exacerbates translational fidelity defects in an rps2 mutant background. Together, our findings reveal how a ribosomal protein N-terminal extension coordinates sequential interactions and post-translational modification events that shape the early fate of Rps2 and support accurate ribosome function.
    Keywords:  Hmt1; Rps2; Saccharomyces cerevisiae; Tsr4; nuclear transport; post‐translational modification (PTM); protein arginine methylation; protein methylation; protein‐protein interaction; ribosome assembly
    DOI:  https://doi.org/10.1016/j.jbc.2026.113481
  4. NAR Genom Bioinform. 2026 Sep;8(3): lqag100
    MetaTIS: a tool to predict cognate and near-cognate translation initiation sites in human.
    Aram Papazian, Volkhard Helms.
      Ribosomes typically commence translation at a methionine-encoding AUG codon flanked by a so-called Kozak region, a short nucleic acid motif that serves as an initiation site in humans. Though, the characteristic AUG start codon of an mRNA is not always effective in initiating translation. Near-cognate codons differing from AUG by one nucleotide may also be recognized as start sites. Several types of ribosomal profiling techniques have been developed that elucidate active translation initiation sites (TIS) that enable training of computational models to predict both cognate and near-cognate TIS using mRNA sequence features. Here, a meta-model termed MetaTIS was implemented by combining outputs of genomic and protein language models fine-tuned on Ensembl annotations of transcripts and five different TIS datasets. The model proficiently differentiates between spurious and true TIS in four distinct test sets, for both AUG and non-AUG instances. Most important for translation initiation based on one of the base model outputs was the Kozak sequence context and a region further upstream in the 5'UTR [-12, -10]. MetaTIS is available as a webserver at https://service2.bioinformatik.uni-saarland.de/metatis/, a tool that accurately predicts TIS for AUG and nine near-cognate start codons.
    DOI:  https://doi.org/10.1093/nargab/lqag100
  5. Nucleic Acids Res. 2026 Aug 24. pii: gkag832. [Epub ahead of print]54(16):
    hY3 RNA binds and inhibits ribosomes and modulates the starvation-induced integrated stress response.
    Valentina Pecoraro, EunBin Kong, Puneet Sharma, Yulia Gonskikh, Marek Żywicki, Fabian Nagelreiter, Markus Schosserer, Katrin Freiburghaus, Jean-Marc Nuoffer, Johannes Grillari, Norbert Polacek.
      Cells adapt to metabolic stress by orchestrating gene expression to mitigate cellular damage, sustain homeostasis, and promote survival. Within this framework, translational control provides a rapid and efficient layer of regulation. Non-coding RNAs have recently emerged as effective modulators of translation, partly by targeting the ribosome. The contribution of ribosome-associated non-coding RNAs (rancRNAs) to translation regulation, however, remains largely unexplored in human cells. Here, we identified the human Y3 (hY3) RNA as a rancRNA that inhibits protein synthesis and attenuates cellular metabolism. hY3 function was particularly critical under nutrient deprivation, where it promoted adaptive stress responses. In this context, depletion of hY3 disrupted the delicate balance between survival and apoptosis by reducing the expression of pro-survival factors and impairing the activation of the integrated stress response (ISR). Loss of hY3 reduced starvation-dependent phosphorylation of eukaryotic translation initiation factor 2α, thereby attenuating ISR signalling, which results in non-physiologically elevated global translation rates during nutrient deprivation. Together, our findings establish hY3 as a ribosome-bound regulator of translation and stress responses, positioning it as a determinant of cell fate under metabolic stress.
    DOI:  https://doi.org/10.1093/nar/gkag832
  6. Cell Rep. 2026 Aug 22. pii: S2211-1247(26)00965-4. [Epub ahead of print]45(9): 117887
    16S ribosomal RNA modification drives transcript-specific translation efficiency.
    Zachory M Park, Christina R Savage, Amanda R Decker-Farrell, Chin-Hsien Tai, Tapan K Maity, Weiming Yang, Lisa M Jenkins, Kumaran S Ramamurthi.
      Bacterial ribosomal RNAs (rRNAs) are decorated with conserved nucleotide modifications, but the functionality of these modifications is often underexplored. MraW (RsmH) is a 16S rRNA methyltransferase. Here, we report that deletion of mraW corrects a late-stage sporulation defect in Bacillus subtilis by bypassing a sporulation checkpoint. Ribosomes purified from ΔmraW cells display a ∼2-fold decrease in translation efficiency; in vivo, ΔmraW cells produced decreased levels of the sporulation checkpoint protein CmpA. Reduced production of CmpA is mediated by mRNA sequences that form a stem-loop which occludes early cmpA codons. Proteomic analysis revealed that MraW mediates production of multiple proteins, some of whose mRNA form similar structures as the cmpA transcript. We propose that MraW modification of 16S rRNA enhances translation efficiency in general, and that specific transcripts have evolved structural features that fine-tune protein levels. This type of control may be prevalent in bacteria which exhibit uncoupled transcription and translation.
    Keywords:  30S subunit; CP: microbiology; CP: molecular biology; RsmI; SpoIVA; SpoVM; elongation; initiation; stationary phase
    DOI:  https://doi.org/10.1016/j.celrep.2026.117887
  7. PLoS Biol. 2026 Aug;24(8): e3003954
    Tcf15 promotes an open nucleolar chromatin state to safeguard ribosome biogenesis and genome stability in mouse embryonic stem cells.
    Yu-Ping Dong, Yi-Min Chen, Min Tang, Hu Zhou, Lin Wang, Ping Zheng.
      Embryonic stem cells (ESCs) exhibit a hyperactive chromatin state at ribosomal RNA (rRNA) genes, which not only plays roles in active rRNA synthesis and ribosome biogenesis (RiBi), but also links to genome architecture. However, how this active chromatin state is maintained in ESCs remains poorly understood. Here, we identify Tcf15, a mouse ESC-specific factor, as a novel regulator of ribosomal DNA (rDNA) chromatin state. Tcf15 localizes to the nucleolus, binds the coding region of rRNA genes, and independently recruits epigenetic modifiers-either Tet2 or Rbbp5 (a core component of H3K4 methyltransferases)-to promote an active chromatin configuration. Depletion of Tcf15 increases DNA methylation and H3K27me3 levels at rDNA. Intriguingly, the Tcf15-Rbbp5 axis ensures precursor rRNA transcription and RiBi, whereas the Tcf15-Tet2 axis is not involved in rRNA synthesis. Ribosome profiling further revealed compromised translation of a subset of mRNAs involved in DNA replication, damage response, and repair. Consequently, Tcf15- or Rbbp5-deficient ESCs exhibit severe genomic instability. Our findings add a new regulatory layer of chromatin state in rDNA of stem cells, and reveal a previously unrecognized phenotypic consequence of defective RiBi in ESCs.
    DOI:  https://doi.org/10.1371/journal.pbio.3003954
  8. RSC Chem Biol. 2026 Aug 20.
    RNA modifications and RNA-binding proteins: interactions, regulation, and new avenues for therapeutic modulation.
    Gabriela Vega-Hernández, Amanda L Garner.
      RNA-binding proteins (RBPs) are central regulators of post-transcriptional gene expression, recognizing RNAs through sequence, structure, and chemical modifications. Post-transcriptional RNA modifications, including m6A, m1A, m5C, m7G, and pseudouridine (Ψ), form the epitranscriptome, a dynamic regulatory layer that modulates RNA stability, localization, and translation. These modifications are interpreted by specialized "reader" RBPs that translate epitranscriptomic marks into functional outcomes. Dysregulation of RNA modifications or their associated reader RBPs has been increasingly linked to the development of cancers, neurological disorders, and other diseases, highlighting their potential for therapeutic manipulation. This review summarizes key RNA modifications and regulating RBPs with a specific emphasis on how dysregulation can lead to cancers. We further discuss current approaches for investigating and manipulating reader RBP-RNA interactions, highlighting how these methods enable new opportunities for therapeutic discovery.
    DOI:  https://doi.org/10.1039/d6cb00136j
  9. Bio Protoc. 2026 Aug 20. 16(16): e5791
    A Luciferase-Based Assay for Assessing Cap-Independent Translation in Wheat Germ Extract.
    Max Cortot, Thorsten Stehlik, Aline Koch, Timo Schlemmer.
      Efficient protein synthesis in eukaryotic cells typically requires a 5' cap structure on messenger RNAs (mRNAs). However, under stress conditions or in viral infection, translation can also occur independently of the cap via internal ribosomal entry sites (IRES). IRES elements are therefore key regulators of protein expression in both viral and cellular contexts. Here, we describe a cell-free protocol to quantitatively assess cap-independent translation using wheat germ extract (WGE) and a firefly luciferase (FLuc) reporter. The protocol includes template preparation, RNA synthesis, and luminescence measurement following in vitro translation in WGE. This method enables rapid and robust comparison of translation activity under controlled conditions and can additionally be applied to evaluate mRNA modifications designed to enhance translation efficiency. Key features • Stringent in vitro workflow from DNA template preparation through RNA synthesis and protein synthesis to reporter readout, including quality controls. • Evaluation of cap-independent translation suitable for testing combinations of IRES and CDS. • Translation analysis without radioactive labeling.
    Keywords:  Cap-independent translation; Firefly luciferase reporter; IRES; In vitro translation; Internal ribosomal entry site; Translation efficiency; Wheat germ extract; mRNA modification
    DOI:  https://doi.org/10.21769/BioProtoc.5791
  10. Front Oncol. 2026 ;16 1863527
    Ribosomal proteins, miRNAs, and extracellular vesicles in myelodysplastic syndromes: molecular regulation and biomarker potential.
    Gazmend Temaj, Sarmistha Saha, Brigitta Buttari, Pelin Telkoparan-Akillilar, Silvia Chichiarelli, Nexhibe Nuhii, Rifat Hadziselimovic, Luciano Saso.
      Myelodysplastic syndromes (MDS) are a heterogeneous group of disorders caused by abnormalities at the hematopoietic stem cell level and are associated with ineffective hematopoiesis, peripheral cytopenias, and a propensity for leukemic transformation. Recent research elucidates the key mechanisms and roles of ribosomal proteins (RPs), microRNAs (miRNAs), extracellular vesicles (EVs), and long non-coding RNAs (lncRNAs) in the pathophysiology of MDS. Mutations or altered expression of RP genes lead to ribosomal stress and activation of p53, resulting in impaired erythropoiesis and apoptosis of hematopoietic progenitors. Dysregulation of miRNAs modulates gene expression programs that are essential for hematopoietic differentiation and apoptosis. Moreover, EV-associated miRNAs mediate multiple processes, including intercellular communication and regulation of the bone marrow (BM) microenvironment, thereby contributing to DNA damage and clonal evolution. EV-associated miR-10a and miR-15a have been reported to induce DNA damage in HSCs. LncRNAs have emerged as promising tools for cancer diagnosis and prognostic biomarkers. They play critical roles in regulating malignant cell proliferation, apoptosis, and epigenetic modifications. This overview highlights the molecular complexity of MDS pathogenesis, offering insights to support stratified diagnosis and the development of targeted therapies.
    Keywords:  MDS; extracellular vesicle; lncRNA; microRNA; ribosomal proteins
    DOI:  https://doi.org/10.3389/fonc.2026.1863527
  11. Trends Cell Biol. 2026 Aug 27. pii: S0962-8924(26)00159-5. [Epub ahead of print]
    Remaking an exit: dynamic regulation of ER exit sites by post-translational modifications.
    Tetsuya Hirata, Michael Boyce.
      Endoplasmic reticulum exit sites (ERES) are dynamic platforms that coordinate not only protein trafficking but also protein quality control and signaling functions. ERES size, composition, architecture, interactions, and activity must be precisely remodeled in response to myriad pathophysiological cues to finely tune cellular homeostasis. However, the molecular mechanisms underlying this regulation remain incompletely understood. Coat protein complex II (COPII) mediates many ERES functions, and its subunits and client cargos are subject to spatiotemporal regulation by post-translational modifications (PTMs) that modulate ERES assembly, cargo selection, and inter-organelle communication. In this article, we review recent advances in understanding how PTMs control the organization and functional versatility of ERES and the COPII system.
    Keywords:  COPII; ER exit site; ER-phagy; post-translational modifications; protein trafficking; signaling
    DOI:  https://doi.org/10.1016/j.tcb.2026.08.003
  12. Proc Natl Acad Sci U S A. 2026 Sep;123(35): e2618832123
    RNA aptamers for sodium and lithium are abundant in mammals.
    Neil White, Gabriel Belem de Andrade, Aya Narunsky, Christopher King, Narasimhan Sudarsan, Ronald R Breaker.
      Riboswitches are common in the bacterial domain of life where they regulate gene expression in response to binding metabolites, elemental ions, or other small ligands. We used comparative sequence analyses to identify numerous RNA motifs in humans and other mammals that are similar in sequence and structure to bacterial riboswitch aptamers for cationic sodium and lithium. Candidates are found in the mRNA transcripts for ~70 genes relevant to ion conductance, neuronal development and function, or to various neurological diseases. Bioinformatic and biochemical analyses support the hypothesis that mammals make extensive use of these RNA aptamers to selectively bind Na+ and Li+. These structured RNA domains are often located in regions of mRNAs or their putative antisense transcripts that suggest they are components of riboswitches. Such associations also expose links between these alkali metal ions and genes whose expression is likely under monovalent ion regulation. These findings are also consistent with the hypothesis that Li+ is a natural contributor to the regulation of genes relevant to certain mental disorders.
    Keywords:  GNPAT; SCN5A; alkali metal; bipolar disorder; riboswitch
    DOI:  https://doi.org/10.1073/pnas.2618832123
  13. Nat Cell Biol. 2026 Aug 25.
    The proportional scaling of mRNA and ribosome concentrations controls eukaryotic cell growth.
    Xin Gao, Michael Lanz, Rosslyn Grosely, Jonas Cremer, Joseph Puglisi, Jan M Skotheim.
      Cell growth underlies nearly all eukaryotic physiology, yet its quantitative principles remain unclear. Here, using single-molecule ribosome tracking, spike-in RNA sequencing and quantitative proteomics across 15 nutrient-limited conditions in budding yeast, we define how growth is controlled in the budding yeast Saccharomyces cerevisiae. Ribosome concentration scales linearly with growth rate, while peptide elongation speed remains constant at approximately nine amino acids per second. While elongation is not a regulatory lever, total mRNA concentration increases proportionally with ribosomes to accelerate growth. A simple kinetic model of mRNA-ribosome binding accurately predicts the fraction of active ribosomes, growth rate and responses to transcriptional or size perturbations. Consistent with this model, transient inhibition of mRNA degradation boosts growth by elevating mRNA concentration. These results reveal that eukaryotic cells accelerate proliferation primarily by proportionally scaling mRNA and ribosome abundance, establishing a quantitative framework for understanding eukaryotic biosynthesis.
    DOI:  https://doi.org/10.1038/s41556-026-02045-0
  14. Front Oncol. 2026 ;16 1891222
    RPLP0 identified as a ferroptosis regulator in HCC through pan-cancer analysis.
    Zuli Wang, Shuai Chen, Bokang Yan, Xintong Peng, Ling Chen, Min Su, Haiyang Li.
       Background: RPLP0, a ribosomal protein critical for protein biosynthesis, has emerged as a multifaceted oncoprotein through its regulation of programmed cell death (PCD) pathways. Despite its established roles in tumorigenesis, its pan-cancer relevance and ferroptosis regulatory function remains unexplored.
    Methods: Bioinformatics analyses utilized TCGA, GTEx, GEPIA, UALCAN, HPA, cBioPortal, TISIDB, FerrDb, BioGRID, HitPredict, and R Studio. Experimental validation in hepatocellular carcinoma (HCC) included RT-qPCR, IHC, CCK8, transwell, and colony formation assays. Ferroptosis was assessed via lipid ROS, ferrous iron, and GSH detection.
    Results: Our comprehensive pan-cancer analysis revealed conserved RPLP0 upregulation across malignancies, positively associated with adverse clinical outcomes. Genomic profiling identified recurrent RPLP0 alterations, while tumor microenvironment analyses demonstrated significant negative correlations between RPLP0 expression and immune cell infiltration. Focusing on hepatocellular carcinoma (HCC), experimental validation confirmed that RPLP0 depletion suppressed tumor progression via ferroptosis induction through metabolic and oxidative cascades, and RPLP0 was positively correlated with key ferroptosis suppressor GPX4.
    Conclusions: This work advances the understanding of ribosomal protein biology by establishing RPLP0 as a pan-cancer biomarker bridging ribosome function with immune evasion, unveiling its canonical role in HCC progression, and discovering RPLP0-GPX4-mediated ferroptosis resistance axis, providing a novel therapeutic paradigm for aggressive cancers.
    Keywords:  GPX4; HCC; RPLP0; ferroptosis; pan-cancer
    DOI:  https://doi.org/10.3389/fonc.2026.1891222
  15. Biol Lett. 2026 Aug 26. pii: 20260144. [Epub ahead of print]22(8):
    Mitochondrial ribosome content as a proxy for respiration.
    Francis Mairet.
      Quantifying cellular activities remains a major challenge across fields ranging from microbial ecology to biotechnology and biomedical sciences. Building on the well-established linear relationship between growth rate and ribosome content-the so-called microbial growth law-this study proposes using organelle ribosome content to infer metabolic activity. In exponentially growing yeast (Saccharomyces cerevisiae), including under overflow metabolism conditions, a strong linear correlation was observed between mitochondrial ribosome content and oxygen uptake rate, underscoring the potential of this approach. Additionally, under fully respiratory conditions, cytoplasmic and mitochondrial ribosome fractions were linearly correlated, whereas overflow conditions fell below this linear relationship, providing a means to identify such metabolic states. Although these findings require broader validation across additional species, organelle ribosome quantification may provide a promising proxy for deciphering cellular metabolism.
    Keywords:  cellular resource allocation; microbial growth laws; overflow metabolism; oxygen uptake rates
    DOI:  https://doi.org/10.1098/rsbl.2026.0144
  16. Sci Adv. 2026 Aug 28. 12(35): eaec9028
    Muscular dystrophy-associated lamin variants disrupt cellular organization through a nucleolar-ribosomal axis.
    Xiangyi Ding, Sweta Kumari, Ellen F Gregory, Daniel A Starr, G W Gant Luxton.
      Emery-Dreifuss muscular dystrophy (EDMD) arises from mutations in nuclear lamins or emerin. Current pathological models emphasize defective nuclear mechanics and transcriptional regulation, yet these mechanisms cannot explain how lamina defects propagate across the cell to produce the complex pathology of laminopathies. Here, we reveal an emerging pathway linking nuclear lamina dysfunction to cytoplasmic reorganization. Using Caenorhabditis elegans EDMD models, we show that disease-linked lamin variants reduce cytoplasmic mesoscale crowding, increase molecular diffusivity, and disrupt nuclear positioning and endoplasmic reticulum architecture, which mirror phenotypes caused by ribosome depletion. Lamin dysfunction also lowers nucleolar fibrillarin levels and ribosome abundance, revealing a nucleolar-ribosomal axis that transmits nuclear defects to the cytoplasm. Loss of the redundant LEM-domain proteins emr-1 and lem-2 phenocopied lamin mutants, indicating that cytoplasmic disorganization is a shared hallmark of EDMD. These findings connect nuclear architecture to whole-cell biophysics and suggest therapeutic strategies aimed at restoring ribosome function.
    DOI:  https://doi.org/10.1126/sciadv.aec9028
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