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Variants in EXOSC9 Disrupt the RNA Exosome and Result in Cerebellar Atrophy with Spinal Motor Neuronopathy.

Burns DT, Donkervoort S, Müller JS, et al. Variants in EXOSC9 Disrupt the RNA Exosome and Result in Cerebellar Atrophy with Spinal Motor Neuronopathy. Am J Hum Genet. 2018;102(5):858-873. doi:10.1016/j.ajhg.2018.03.011

Resolving cell state in iPSC-derived human neural samples with multiplexed fluorescence imaging.

Tomov ML, O’Neil A, Abbasi HS, et al. Resolving cell state in iPSC-derived human neural samples with multiplexed fluorescence imaging. Commun Biol. 2021;4(1):786. doi:10.1038/s42003-021-02276-x

Reactive Astrocytes Promote ALS-like Degeneration and Intracellular Protein Aggregation in Human Motor Neurons by Disrupting Autophagy through TGF-β1.

Tripathi P, Rodriguez-Muela N, Klim JR, et al. Reactive Astrocytes Promote ALS-like Degeneration and Intracellular Protein Aggregation in Human Motor Neurons by Disrupting Autophagy through TGF-β1. Stem Cell Reports. 2017;9(2):667-680. doi:10.1016/j.stemcr.2017.06.008

Modeling ALS with motor neurons derived from human induced pluripotent stem cells.

Sances S, Bruijn LI, Chandran S, et al. Modeling ALS with motor neurons derived from human induced pluripotent stem cells. Nat Neurosci. 2016;19(4):542-53. doi:10.1038/nn.4273

Motoneurons derived from induced pluripotent stem cells develop mature phenotypes typical of endogenous spinal motoneurons.

Toma JS, Shettar BC, Chipman PH, et al. Motoneurons derived from induced pluripotent stem cells develop mature phenotypes typical of endogenous spinal motoneurons. J Neurosci. 2015;35(3):1291-306. doi:10.1523/JNEUROSCI.2126-14.2015

Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing mutations.

Alami NH, Smith RB, Carrasco MA, et al. Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing mutations. Neuron. 2014;81(3):536-543. doi:10.1016/j.neuron.2013.12.018

How to make spinal motor neurons.

Davis-Dusenbery BN, Williams LA, Klim JR, Eggan K. How to make spinal motor neurons. Development. 2014;141(3):491-501. doi:10.1242/dev.097410

Changes in the Excitability of Neocortical Neurons in a Mouse Model of Amyotrophic Lateral Sclerosis Are Not Specific to Corticospinal Neurons and Are Modulated by Advancing Disease.

Kim J, Hughes EG, Shetty AS, et al. Changes in the Excitability of Neocortical Neurons in a Mouse Model of Amyotrophic Lateral Sclerosis Are Not Specific to Corticospinal Neurons and Are Modulated by Advancing Disease. J Neurosci. 2017;37(37):9037-9053. doi:10.1523/JNEUROSCI.0811-17.2017

Pathways disrupted in human ALS motor neurons identified through genetic correction of mutant SOD1.

Kiskinis E, Sandoe J, Williams LA, et al. Pathways disrupted in human ALS motor neurons identified through genetic correction of mutant SOD1. Cell Stem Cell. 2014;14(6):781-95. doi:10.1016/j.stem.2014.03.004

Intrinsic membrane hyperexcitability of amyotrophic lateral sclerosis patient-derived motor neurons.

Wainger BJ, Kiskinis E, Mellin C, et al. Intrinsic membrane hyperexcitability of amyotrophic lateral sclerosis patient-derived motor neurons. Cell Rep. 2014;7(1):1-11. doi:10.1016/j.celrep.2014.03.019