UPF1 Reduces C9orf72 HRE-Induced Neurotoxicity in the Absence of Nonsense-Mediated Decay Dysfunction

Scientists investigated the role that overexpression of UPF1, a protein involved in nonsense-mediated decay, plays in mitigating neurotoxicity in multiple models of C9orf72 ALS/FTD.
[Cell Reports]
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Adipose Derived Stem Cells Protect Motor Neurons and Reduce Glial Activation in both In Vitro and In Vivo Models of ALS

Researchers tested the in vitro neuroprotective potential of mouse adipose derived stem cells in astrocyte/motor neuron co-cultures where amyotrophic lateral sclerosis (ALS) astrocytes show neurotoxicity.
[Molecular Therapy-Methods & Clinical Development]
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A Microfabricated Multi-Compartment Device for Neuron and Schwann Cell Differentiation

Scientists report a multi-compartment microfluidic device where up to three different cell populations could be cultured in a fluidically independent circuit. The device allows cell migration across the compartments and their differentiation.
[Scientific Reports]
De Vitis, E., La Pesa, V., Gervaso, F., Romano, A., Quattrini, A., Gigli, G., Moroni, L., & Polini, A. (2021). A microfabricated multi-compartment device for neuron and Schwann cell differentiation. Scientific Reports, 11(1), 7019. https://doi.org/10.1038/s41598-021-86300-4 Cite
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Retinal Glial Remodeling by FGF21 Preserves Retinal Function during Photoreceptor Degeneration

Single-cell transcriptomics of retinal cells, showed that fibroblast growth factor 21 (FGF21) receptor Fgfr1 was specifically expressed in Müller glia/astrocytes.
[iScience]
Fu, Z., Qiu, C., Cagnone, G., Tomita, Y., Huang, S., Cakir, B., Kotoda, Y., Allen, W., Bull, E., Akula, J. D., Joyal, J.-S., Hellström, A., Talukdar, S., & Smith, L. E. H. (2021). Retinal glial remodeling by FGF21 preserves retinal function during photoreceptor degeneration. IScience, 0(0). https://doi.org/10.1016/j.isci.2021.102376 Cite
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Sensing Serotonin Secreted from Human Serotonergic Neurons Using Aptamer-Modified Nanopipettes

Investigators generated human serotonergic neurons in vitro and detected secreted serotonin using aptamer-coated nanopipettes in a low nanomolar range, with the possibility of detecting significantly lower concentrations.
[Molecular Psychiatry]
Nakatsuka, N., Heard, K. J., Faillétaz, A., Momotenko, D., Vörös, J., Gage, F. H., & Vadodaria, K. C. (2021). Sensing serotonin secreted from human serotonergic neurons using aptamer-modified nanopipettes. Molecular Psychiatry, 1–11. https://doi.org/10.1038/s41380-021-01066-5 Cite
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The Difficulty to Model Huntington’s Disease In Vitro Using Striatal Medium Spiny Neurons Differentiated from Human Induced Pluripotent Stem Cells

Researchers compared two previously published protocols to obtain human induced pluripotent stem cell-derived striatal neurons from both healthy donors and Huntington’s disease patients.
[Scientific Reports]
Le Cann, K., Foerster, A., Rösseler, C., Erickson, A., Hautvast, P., Giesselmann, S., Pensold, D., Kurth, I., Rothermel, M., Mattis, V. B., Zimmer-Bensch, G., von Hörsten, S., Denecke, B., Clarner, T., Meents, J., & Lampert, A. (2021). The difficulty to model Huntington’s disease in vitro using striatal medium spiny neurons differentiated from human induced pluripotent stem cells. Scientific Reports, 11(1), 6934. https://doi.org/10.1038/s41598-021-85656-x Cite
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Generation of UCiPSC-Derived Neurospheres for Cell Therapy and Its Application

Scientists provide a simple method for the storage of neural stem cells as neurospheres at ambient temperature as an alternative method to more costly and inconvenient traditional methods of cryopreservation.
[Stem Cell Research & Therapy]
Li, S., Zhao, H., Han, X., Ni, B., He, L., Mukama, O., de Dieu Habimana, J., Lin, Z., Huang, R., Huang, H., Tian, C., Tang, F., & Li, Z. (2021). Generation of UCiPSC-derived neurospheres for cell therapy and its application. Stem Cell Research & Therapy, 12(1), 188. https://doi.org/10.1186/s13287-021-02238-4 Cite
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Establishment of an In Vitro Model for Analyzing Mitochondrial Ultrastructure in PRKN-Mutated Patient iPSC-Derived Dopaminergic Neurons

To selectively label dopaminergic neurons and analyze mitochondrial morphology at the ultrastructural level, investigators generated control and PRKN-mutated patient tyrosine hydroxylase reporter iPSC lines.
[Molecular Brain]
Establishment of an in vitro model for analyzing mitochondrial ultrastructure in PRKN -mutated patient iPSC-derived dopaminergic neurons | Molecular Brain | Full Text. (n.d.). Retrieved March 24, 2021, from https://molecularbrain.biomedcentral.com/articles/10.1186/s13041-021-00771-0# Cite
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The Role of Mitophagy in the Regulation of Mitochondrial Energetic Status in Neurons

Investigators revealed that mitophagy was broadly activated in metabolically enhanced neurons upon oxidative phosphorylation stimulation, which sustained high energetic activity by increasing mitochondrial turnover and hence facilitated mitochondrial maintenance.
[Autophagy]
Han, S., Zhang, M., Jeong, Y. Y., Margolis, D., & Cai, Q. (2021). The Role of Mitophagy in the Regulation of Mitochondrial Energetic Status in Neurons. Autophagy, 0(ja), null. https://doi.org/10.1080/15548627.2021.1907167 Cite
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The DJ1-Nrf2-STING Axis Mediates the Neuroprotective Effects of Withaferin A in Parkinson’s Disease

The authors showed that Withaferin A protected against loss of dopaminergic neurons, neuroinflammation, and motor deficits in MPTP-induced Parkinson’s disease mouse models.
[Cell Death & Differentiation]
Zhao, M., Wang, B., Zhang, C., Su, Z., Guo, B., Zhao, Y., & Zheng, R. (2021). The DJ1-Nrf2-STING axis mediates the neuroprotective effects of Withaferin A in Parkinson’s disease. Cell Death & Differentiation, 1–19. https://doi.org/10.1038/s41418-021-00767-2 Cite
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Increased LRRK2 Kinase Activity Alters Neuronal Autophagy by Disrupting the Axonal Transport of Autophagosomes

Leucine-rich repeat kinase 2 (LRRK2)-G2019S, dramatically reduces the processivity of autophagosome transport in neurons in a kinase-dependent manner. This effect was consistent across an overexpression model, neurons from a G2019S knockin mouse, and human induced pluripotent stem cell-derived neurons gene edited to express the G2019S mutation, and the effect was reversed by genetic or pharmacological inhibition of LRRK2.
[Current Biology]
Boecker, C. A., Goldsmith, J., Dou, D., Cajka, G. G., & Holzbaur, E. L. F. (2021). Increased LRRK2 kinase activity alters neuronal autophagy by disrupting the axonal transport of autophagosomes. Current Biology, 0(0). https://doi.org/10.1016/j.cub.2021.02.061 Cite
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