Summary: Animal models have greatly facilitated the process of drug discovery. Len Zon draws on his own experiences to highlight how a cross-model, interdisciplinary approach can bring basic findings to the clinic.
Summary: Reproducibility of findings in experiments using model organisms has recently become a source of concern, particularly for translational science. We discuss factors affecting the interpretation and reliability of experimental pathology findings in the mouse, and how disclosure and transparent reporting are crucial for replicability.
Musculocontractural Ehlers–Danlos syndrome and neurocristopathies: dermatan sulfate is required for Xenopus neural crest cells to migrate and adhere to fibronectin
Editors' choice: In the Xenopus neural crest, dermatan sulfate is essential for cell migration in vivo and cell adhesion to fibronectin, which might have implications for musculocontractural Ehlers–Danlos syndrome and cancer.
Identification of benzopyrone as a common structural feature in compounds with anti-inflammatory activity in a zebrafish phenotypic screen
Summary: Zebrafish inflammation screen identifies a new series of structurally related compounds with combined anti-inflammatory and pro-resolution activity, and reveals a previously unknown mechanism of action of clinical cromones.
Summary: We have assessed the effect of the epigenetic post-translational modification histone crotonylation during kidney injury in vivo and in cell culture, and the involvement of PGC-1α and SIRT3 in the process.
Two different pathogenic mechanisms, dying-back axonal neuropathy and pancreatic senescence, are present in the YG8R mouse model of Friedreich’s ataxia
Summary: Frataxin deficiency induces different pathogenic mechanisms in the nervous system and pancreas in a YG8R mouse model of Friedreich's ataxia (FRDA). Thus, the degenerative process in FRDA is determined by the cell type.
A novel Drosophila model of TDP-43 proteinopathies: N-terminal sequences combined with the Q/N domain induce protein functional loss and locomotion defects
Summary: An engineered TDP-43 construct can be used to induce TDP-43 aggregation in Drosophila, providing a model that could be useful for characterization of pathogenetic mechanisms and drug screening.
Cellular dynamics of regeneration reveals role of two distinct Pax7 stem cell populations in larval zebrafish muscle repair
Summary: Tracking muscle precursor cells during wound repair with confocal time-lapse microscopy reveals that two myoblast populations fuse and contribute differentially to regeneration, one initiating and the other growing fibres.
High-throughput screening for modulators of ACVR1 transcription: discovery of potential therapeutics for fibrodysplasia ossificans progressiva
Summary: We describe the identification of dipyridamole as a potential therapeutic tool for FOP, through a series of in vitro and in vivo assays to screen and validate FDA-approved compounds.
Muscles provide protection during microbial infection by activating innate immune response pathways in Drosophila and zebrafish
Summary: Using fruit fly and zebrafish models, we show that skeletal muscles are immune responsive tissues; they mount innate immune responses during bacterial infection – an evolutionarily conserved defense mechanism.
TLR-mediated albuminuria needs TNFα-mediated cooperativity between TLRs present in hematopoietic tissues and CD80 present on non-hematopoietic tissues in mice
Summary: Systemic TNFα mediates myeloid cell and podocyte cross-talk to cause LPS-induced mouse microalbuminuria, a partial model of human nephrotic syndrome, pointing to potential adjunct therapeutic approaches.