Functional and Molecular Properties of DYT-SGCE Myoclonus-Dystonia Patient-Derived Striatal Medium Spiny Neurons

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]
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
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Integrins Protect Sensory Neurons in Models of Paclitaxel-Induced Peripheral Sensory Neuropathy

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]
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
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Targeting Autophagy as a Therapeutic Strategy to Prevent Dopamine Neuron Loss in Early Stages of Alzheimer Disease

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.
[Autophagy]
Nobili, A., Barbera, L. L., & D’Amelio, M. (2021). Targeting autophagy as a therapeutic strategy to prevent dopamine neuron loss in early stages of Alzheimer disease. Autophagy, 0(0), 1–3. https://doi.org/10.1080/15548627.2021.1909409 Cite
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Astrocytes Close a Motor Circuit Critical Period

Scientists defined a critical period in a developing Drosophila motor circuit and identified astrocytes as essential for proper critical period termination.
[Nature]
Ackerman, S. D., Perez-Catalan, N. A., Freeman, M. R., & Doe, C. Q. (2021). Astrocytes close a motor circuit critical period. Nature, 1–7. https://doi.org/10.1038/s41586-021-03441-2 Cite
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The Autism-Associated Protein CHD8 Is Required for Cerebellar Development and Motor Function

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.
[Cell Reports]
Kawamura, A., Katayama, Y., Kakegawa, W., Ino, D., Nishiyama, M., Yuzaki, M., & Nakayama, K. I. (2021). The autism-associated protein CHD8 is required for cerebellar development and motor function. Cell Reports, 35(1). https://doi.org/10.1016/j.celrep.2021.108932 Cite
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Novel Genome-Editing-Based Approaches to Treat Motor Neuron Diseases: Promises and Challenges

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.
[Molecular Therapy]
Miccio, A., Antoniou, P., Ciura, S., & Kabashi, E. (2021). Novel genome-editing-based approaches to treat motor neuron diseases: promises and challenges. Molecular Therapy, 0(0). https://doi.org/10.1016/j.ymthe.2021.04.003 Cite
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Ionis Initiates Phase III Trial of Novel Antisense Medicine to Treat Leading Cause of Juvenile-Onset ALS

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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Incorporation of a Nucleoside Analog Maps Genome Repair Sites in Postmitotic Human Neurons

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.
[Science]
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Dynamic Spatiotemporal Coordination of Neural Stem Cell Fate Decisions Occurs through Local Feedback in the Adult Vertebrate Brain

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 Human Induced Pluripotent Stem Cell-Derived Neural Crest Cells for Corneal Endothelial Regeneration

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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ATF5 Deficiency Causes Abnormal Cortical Development

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.
[Scientific Reports]
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