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EDITORIAL

Summary: This Editorial provides a brief historical perspective on the transition of the nematode roundworm, Caenorhabditis elegans, from a purely basic research organism to a preclinical model for human disease.

OBITUARY

A MODEL FOR LIFE

PERSPECTIVE

Summary: Organ-on-a-chip devices, which combine engineered 3D skeletal muscle tissues and integrated biosensors, hold great promise as a tool for studying muscular dystrophies and developing new therapies for these conditions.

REVIEWS

Summary: Advances in the study of the cylindromatosis gene (CYLD), utilising diverse models, provide new insights into cellular homeostasis and human diseases.

Summary: This Review summarizes and promotes the use of Caenorhabditis elegans to investigate ten of the 14 cancer hallmarks that define the mechanisms of cancer.

EDITOR'S CHOICE

RESEARCH ARTICLES

REVIEW COMMONS TRANSFER

Summary: Loss of NR2F1, which regulates mitochondrial genes in neurons, leads to mitochondrial dysfunction and impaired neuron function, which could contribute to pathogenesis of the neurodevelopmental disorder Bosch-Boonstra-Schaaf optic atrophy syndrome.

Summary: LY2090314 suppresses 2D and 3D adipogenesis of fibro/adipogenic progenitors by stabilizing a transcriptional-competent β-catenin complex.

Summary: Kidney-on-a-chip-based in vitro models have greatly evolved and provide valuable instruments to study human kidney (patho-)physiology, contributing to the Reduction, Replacement and Refinement of animal experiments.

Summary: The C921Y O-GlcNAc transferase variant found in patients with intellectual disability leads to a defect in pluripotent stem cell self-renewal and decreased levels of pluripotent stem cell markers.

Summary: This study shows the effectiveness of irinotecan, eribulin, trabectedin and their combination in a patient-derived xenograft model of desmoplastic small round cell tumor, a rare and incurable malignancy.

Summary: Male mice lacking microRNA-29a/b1 develop bladder enlargement and diminished bladder contractility, associated with upregulation of genes related to extracellular matrix formation and turnover.

Summary: Mutations in the renin leader peptide or pro-segment associated with autosomal dominant tubulointerstitial kidney disease lead to misrouting of the mutated protein to mitochondria.

Summary: A novel technique using ultrasound-stimulated microbubbles coupled with fractionated radiation enhanced vascular disruption in the tumour microenvironment via an ASMase-dependent mechanism.

Editor's choice: Activation of R-spondin 1 signaling in the adrenal cortex leads to a sexually dimorphic phenotype in mice, causing hyperplasia in females and immune cell recruitment in males.

Summary: Simultaneous inhibition of gba1 and smpd1 gene function (both implicated in Parkinson's disease) unexpectedly rescues neuronal gene expression and mitochondrial defects, and extends the lifespan of gba1 mutant zebrafish.

Summary: Vemurafenib partially improves muscle histopathology by restoring the TGF-β/SMAD3 and mTORC1/p70S6K signaling pathways but does not improve muscle function in a mouse model of LAMA2-related-congenital muscular dystrophy.

Summary: Surgical rescue of Ednrb−/− rats, a model for Hirschsprung disease, required dietary intervention for survival and showed reduced colon diameter in the aganglionic region, overall normal histology and changes in innervation.

RESOURCE ARTICLES

Summary: Hypokalemic periodic paralysis model cells that allow the fluorometric evaluation of gating pore currents in a multi-well-plate format.

Summary: We developed the deCLUTTER2+ pipeline to analyze calcium traces to discover spontaneous or cue-dependent patterns of Ca2+ transients, and a protocol for the generation of iPSC-derived ventral midbrain patterned astrocytes.

Summary: We report a method to rapidly analyze craniofacial development in zebrafish embryos using morphometric analysis, creating a new platform to quantitatively detect phenotypic variation after genetic alteration.

Summary: Development of an automated image analysis workflow to enable fast and reliant immune cell counting in Shigella-infected zebrafish larvae, and to reveal the in vivo impact of antibiotics on Shigella–neutrophil interactions.

Summary: A new zebrafish transgenic line that labels the expression of the gene arg2 can be used to visualise immune cell polarisation and investigate the mechanisms of innate immunity during infections.

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