To elucidate the underlying pathogenic mechanisms of myoclonus dystonia, scientists investigated iPSC-derived striatal medium spiny neurons from two myoclonus-dystonia patients carrying a heterozygous mutation in the SGCE gene in comparison to two matched healthy control lines.
[International Journal of Molecular Sciences]
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Kutschenko, A., Staege, S., Grütz, K., Glaß, H., Kalmbach, N., Gschwendtberger, T., Henkel, L. M., Heine, J., Grünewald, A., Hermann, A., Seibler, P., & Wegner, F. (2021). Functional and Molecular Properties of DYT-SGCE Myoclonus-Dystonia Patient-Derived Striatal Medium Spiny Neurons. International Journal of Molecular Sciences, 22(7), 3565. https://doi.org/10.3390/ijms22073565 Cite
The authors used a genetically tractable Drosophila model and primary sensory neurons isolated from adult mouse to examine the mechanisms underlying chemotherapy-induced peripheral neuropathy and identify protective pathways.
[Proceedings of the National Academy of Sciences of the United States of America]
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Shin, G. J., Pero, M. E., Hammond, L. A., Burgos, A., Kumar, A., Galindo, S. E., Lucas, T., Bartolini, F., & Grueber, W. B. (2021). Integrins protect sensory neurons in models of paclitaxel-induced peripheral sensory neuropathy. Proceedings of the National Academy of Sciences, 118(15). https://doi.org/10.1073/pnas.2006050118 Cite
Investigators summarized new data showing how macroautophagy/autophagy impairment – due to enhanced activity of the ABL/c-Abl kinase – might have caused the dopamine neuron loss.
Scientists defined a critical period in a developing Drosophila motor circuit and identified astrocytes as essential for proper critical period termination.
The authors showed that cerebellar granule neuron progenitor-specific deletion of chromodomain helicase DNA-binding protein 8 (CHD8) in mice impaired the proliferation and differentiation of the cells as well as gave rise to cerebellar hypoplasia and a motor coordination defect, but not to autism spectrum disorder-like behavioral abnormalities.
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Scientists review genome editing approaches aimed either at correcting the disease-causing mutations or at modulating the expression of genetic modifiers, e.g. by repairing SOD1 mutations or SMN2 splicing defect, or deleting C9orf72 expanded repeats.
Ionis Pharmaceuticals, Inc. announced the initiation of a Phase III clinical trial of ION363 in patients with amyotrophic lateral sclerosis (ALS) with mutations in the fused in sarcoma gene.
[Ionis Pharmaceuticals, Inc.]
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A targeted sequencing approach in human embryonic stem cell–induced neurons showed that, in neurons, DNA repair is enriched at well-defined hotspots that protect essential genes.
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The authors combined dynamic imaging of entire neural stem cell (NSC) populations in their in vivo niche over several weeks with pharmacological manipulations, mathematical modeling, and spatial statistics and demonstrated that NSCs use spatiotemporally resolved local feedback signals to coordinate their decision to divide in adult zebrafish brains.
[Cell Stem Cell]
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Transplantation of the hiPSC-derived neural crest cells rapidly restored rabbit corneal thickness and clarity. However, the long-term recovery efficacy was impaired by the improper maturation, senescence, and endothelial-mesenchymal transition of the transplanted cells.
[Stem Cell Research & Therapy]
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To investigate the cause of abnormal behaviors exhibited by ATF5−/− mice, researchers analyzed the embryonic cerebral cortex of ATF5−/− mice. The ATF5−/− embryonic cerebral cortex was slightly thinner and had reduced numbers of radial glial cells and neural progenitor cells, compared to a wild-type cerebral cortex.
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