Neuroscience
Short-duration splice promoting compound enables a tunable mouse model of spinal muscular atrophyWe describe drug treatment paradigms that allow investigation of cellular and molecular pathogenesis at different stages of spinal muscular atrophy in a mouse model.
Multiplexed chemogenetics in astrocytes and motoneurons restore blood–spinal cord barrier in ALSChemogenetic motoneuron excitation and astrocyte GPCR-Gi signaling restore blood–spinal cord barrier, disrupted in four ALS mouse models, revealing its role in disease progression but not initiation.
Mitochondrial spongiotic brain disease: astrocytic stress and harmful rapamycin and ketosis effectAstrocyte-specific mtDNA depletion causes spongiotic encephalopathy, aggravated by ketogenic diet or rapamycin. Astrocytes, but not neurons, drive mitochondrial integrated stress response in the CNS.
Prion infection, transmission, and cytopathology modeled in a low-biohazard human cell lineExpanding the toolbox of prion research to a low-biohazard, scalable human cell model.
Genetic targeting of neurogenic precursors in the adult forebrain ventricular epitheliumIn vivo evidence for precursors that produce neurons independent of neurosphere-forming neural stem cells suggests the adult forebrain, like the developing brain, has two distinct neurogenic pathways.
Regulation of axonal morphogenesis by the mitochondrial protein Efhd1Ablation of the mitochondrial protein Efhd1 in mice interferes with normal development of sensory axons and results in mitochondrial dysfunction associated with activation of cellular stress pathways.
Importance of γ-secretase in the regulation of liver X receptor and cellular lipid metabolismInhibition of the Alzheimer associated γ-secretase impairs the regulation of cellular lipid droplet homeostasis.
SARM1 deficiency promotes rod and cone photoreceptor cell survival in a model of retinal degenerationThis study reports that activation of SARM1 causes destruction of NAD pools in photoreceptor cells of the retina and identifies a role for SARM1-dependent photoreceptor cell death in retinal degeneration.
Robust production of uniform human cerebral organoids from pluripotent stem cellsBy leveraging advances in 3D stem cell culture techniques, the authors describe, characterize and validate a novel platform to efficiently generate morphologically consistent human forebrain cerebral organoids.
Cx3cr1-deficient microglia exhibit a premature aging transcriptomeGyoneva et al use RNA-seq to show that Cx3cr1-deficient microglia in young mice display a gene expression profile similar to microglia in aged mice, suggesting premature microglial aging.