Summary: The 200th anniversary of the birth of Louis Pasteur is an opportunity to revisit the origins of microbiology and to consider Pasteur's impact on our ability to combat infectious disease.
A MODEL FOR LIFE
Collaboration and knowledge integration for successful brain therapeutics – lessons learned from the pandemic
Summary: This article summarizes the key points and action items that emerged from a virtual Keystone Symposium on advancing brain therapeutics.
Variable phenotypes and penetrance between and within different zebrafish ciliary transition zone mutants
Summary: Comprehensive morphological and molecular analysis of zebrafish transition zone mutants reveals differential roles of these proteins, as well as variable phenotypes within a clutch reminiscent of variability in ciliopathy patients.
Sterol dysregulation in Smith–Lemli–Opitz syndrome causes astrocyte immune reactivity through microglia crosstalk
Summary: Inhibition of cholesterol biosynthesis in microglia results in reactive microgliosis and subsequently astrogliosis in a mouse model of Smith–Lemli–Opitz syndrome, highlighting the role of lipid metabolism in regulating nervous system health and function.
An induced pluripotent stem cell-based model identifies molecular targets of vincristine neurotoxicity
Summary: We established a platform of neurodegeneration with iPSC-derived sensory neurons and investigated the connection between neurotoxicity and signaling pathways. Our results provide the potential for therapeutic targets in vincristine-induced neurodegeneration.
17q12 deletion syndrome mouse model shows defects in craniofacial, brain and kidney development, and glucose homeostasis
Summary: We developed a mouse model of 17q12 deletion syndrome, which phenocopies many of the features of the human disorder, including renal dysfunction, craniofacial, glucose homeostasis and brain abnormalities.
Lysosomal alterations and decreased electrophysiological activity in CLN3 disease patient-derived cortical neurons
Summary: We used proteomics and electrophysiology to investigate how the common 1 kb deletion in CLN3 affects induced pluripotent stem cell-derived neuronal functions, and suggest pathways that may point to new therapeutic opportunities.
Serum inflammatory cytokines as disease biomarkers in the DE50-MD dog model of Duchenne muscular dystrophy
Summary: An evaluation of the serum inflammatory cytokine profile of the newly established DE50-MD dog model of Duchenne muscular dystrophy revealed potential biomarkers for future pre-clinical trials.
Bortezomib-induced neurotoxicity in human neurons is the consequence of nicotinamide adenine dinucleotide depletion
Summary: Human stem cell-derived neurons confirm a role for Wallerian-like degeneration in bortezomib-induced axonal neurotoxicity.
Summary: We report investigation of a new Down syndrome (DS) mouse model, Ts66Yah, with stronger construct and face validity for mimicking consequences of DS genetic overdosage than the premier (Ts65Dn) mouse model of DS.
Microvesicle release from inner segments of healthy photoreceptors is a conserved phenomenon in mammalian species
Summary: Healthy photoreceptor cells release extracellular vesicles directly from their inner segment compartment. This process appears to be amplified in some photoreceptor degenerative models for disposal of mistrafficked proteins.
Editor's choice: Experimental imaging setup, materials and procedures for in vivo and longitudinal confocal imaging of the transparent adult vertebrate model Danionella cerebrum.