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.
A MODEL FOR LIFE
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.
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.
NR2F1 shapes mitochondria in the mouse brain, providing new insights into Bosch-Boonstra-Schaaf optic atrophy syndrome
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.
A 3D adipogenesis platform to study the fate of fibro/adipogenic progenitors in muscular dystrophies
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.
An O-GlcNAc transferase pathogenic variant linked to intellectual disability affects pluripotent stem cell self-renewal
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.
Effectiveness of irinotecan plus trabectedin on a desmoplastic small round cell tumor patient-derived xenograft
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.
Leader peptide or pro-segment mutants of renin are misrouted to mitochondria in autosomal dominant tubulointerstitial kidney disease
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.
Ultrasound-stimulated microbubbles enhanced vascular disruption in fractionated radiotherapy-treated tumours via ASMase activation
Summary: A novel technique using ultrasound-stimulated microbubbles coupled with fractionated radiation enhanced vascular disruption in the tumour microenvironment via an ASMase-dependent mechanism.
Crosstalk between androgen receptor and WNT/β-catenin signaling causes sex-specific adrenocortical hyperplasia in mice
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.
Unexpected phenotypic and molecular changes of combined glucocerebrosidase and acid sphingomyelinase deficiency
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.
Vemurafenib improves muscle histopathology in a mouse model of LAMA2-related congenital muscular dystrophy
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.
Development of the aganglionic colon following surgical rescue in a cell therapy model of Hirschsprung disease in rat
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.
Summary: Hypokalemic periodic paralysis model cells that allow the fluorometric evaluation of gating pore currents in a multi-well-plate format.
deCLUTTER2+ – a pipeline to analyze calcium traces in a stem cell model for ventral midbrain patterned astrocytes
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.
Facial analytics based on a coordinate extrapolation system (zFACE) for morphometric phenotyping of developing zebrafish
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.
An automated microscopy workflow to study Shigella–neutrophil interactions and antibiotic efficacy in vivo
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.