Issues

Summary: This Editorial considers how knowledge from animal and other models of infectious disease can impact our understanding of human biology and potential therapies, focusing largely on zebrafish. It also highlights ways in which DMM is supporting these areas.

Summary: We summarize the consensus guidelines on the construct validity of rodent models of restless legs syndrome, which have been recently established by a task force of the International Restless Legs Syndrome Study Group.

Summary: Neurofibromin influences neural circuits through RAS, cAMP and dopamine signaling. Exploring the mechanisms underlying neurofibromin-dependent behaviors in animal models might enable future treatment of the various cognitive deficits that are associated with neurofibromatosis type 1.

Summary: The hSOD1-G93A mouse, a motor neuron disease model, shows an atypical profile of molecular traits of cell senescence in the lumbar spinal cord, with selective sensitivity to senolytic treatments.

Summary: Daxx and Atrx are not robust tumor suppressors in murine endocrine pancreas, suggesting that the impact of human-specific features (telomere length, transposable elements) on tumorigenesis is not easily recapitulated in mice.

Editor's choice: Exposure of vocal fold mucosae to e-cigarette vapor extract induces lipid-mediated epithelial injury that disrupts vocal fold mucosal homeostasis and innate barrier function and triggers intense mucosal remodeling.

Summary: This article demonstrates the essential role FGF8–FGFR1 signaling has in the development of gonadotropin-releasing hormone (GnRH)-expressing neurons by using a human stem cell model.

Summary: Using an avian model, we report a novel molecular mechanism (disruptions in the FGF23–PTH axis resulting in reduced calcium uptake in the developing mandible) and potential treatment for ciliopathic micrognathia.

Summary: Orthologous neuroblastoma patient-derived point mutations in the Drosophila Anaplastic lymphoma kinase receptor tyrosine kinase drive aberrant signaling, resulting in decreased neuronal apoptosis and cell fate changes.

Summary: In a mouse model of spinocerebellar ataxia type 3 (SCA3), aberrant cerebellar physiology is apparent early in disease, prior to cerebellar neuronal pathology. Aberrant cerebellar output could be a therapeutic target in SCA3.

Summary: We describe the first animal model of Spitz neoplasms and demonstrate its use for modeling sequential mutagenesis and its potential for studying melanocyte development in vivo.

Summary: We have developed a genetic labeling system in zebrafish to enable high-resolution in vivo imaging of dendritic spine dynamics during larval development.

Summary: By using complementary metabolomics technologies, we describe a multidimensional workflow to image the biodistribution and measure the pharmacodynamics of a drug in the adult zebrafish.