A Stem Cell-Based Screening Platform Identifies Compounds that Desensitize Motor Neurons to Endoplasmic Reticulum Stress

Copper-Zinc Superoxide Dismutase (SOD1) Is Released by Microglial Cells and Confers Neuroprotection against 6-OHDA Neurotoxicity

Polazzi, E., Mengoni, I., Caprini, M., Peña‐Altamira, E., Kurtys, E., & Monti, B. (2012) · Neurosignals · doi:10.1159/000337115 · Cited by 9,023 · see citing works

A robust and high-throughput Cre reporting and characterization system for the whole mouse brain

Madisen, L., Zwingman, T. A., Sunkin, S. M., Oh, S. W., Zariwala, H. A., Gu, H., Ng, L. L., Palmiter, R. D., Hawrylycz, M. J., Jones, A. R., et al. (2010) · Nature Neuroscience · doi:10.1038/nn.2467 · Cited by 6,907 · see citing works

Motor Neuron Degeneration in Mice that Express a Human Cu,Zn Superoxide Dismutase Mutation

Gurney, M. E., Pu, H., Chiu, A. Y., Dal Canto, M. C., Polchow, C. Y., Alexander, D. D., Caliendo, J., Hentati, A., Kwon, Y. W., Deng, H.-X., et al. (1994) · Science · doi:10.1126/science.8209258 · Cited by 4,143 · see citing works

The unfolded protein response: controlling cell fate decisions under ER stress and beyond

Hetz, C. A. (2012) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm3270 · Cited by 4,096 · see citing works

Mutation of the mouse klotho gene leads to a syndrome resembling ageing

Kuro-o, M., Matsumura, Y., Aizawa, H., Kawaguchi, H., Suga, T., Utsugi, T., Ohyama, Y., Kurabayashi, M., Kaname, T., Kume, E., et al. (1997) · Nature · doi:10.1038/36285 · Cited by 3,837 · see citing works

Coupling of stress in the ER to activation of JNK protein kinases by transmembrane protein kinase IRE1.

Urano, F., Wang, X.-Z., Bertolotti, A., Zhang, Y.-H., Chung, P., Harding, H., & Ron, D. (2000) · Science · doi:10.1126/science.287.5453.664 · Cited by 3,081 · see citing works

The integrated stress response

Pakos‐Zebrucka, K., Koryga, I., Mnich, K., Ljujic, M., Samali, A., & Gorman, A. M. (2016) · The EMBO Reports · doi:10.15252/embr.201642195 · Cited by 2,659 · see citing works

Chemical Chaperones Reduce ER Stress and Restore Glucose Homeostasis in a Mouse Model of Type 2 Diabetes

Özcan, U., Yilmaz, E., Özcan, L., Furuhashi, M., Vaillancourt, E., Smith, R. O., Görgün, C. Z., & Hotamisligil, G. S. (2006) · Science · doi:10.1126/science.1128294 · Cited by 2,413 · see citing works

Induced Pluripotent Stem Cells Generated from Patients with ALS Can Be Differentiated into Motor Neurons

Dimos, J. T., Rodolfa, K. T., Niakan, K. K., Weisenthal, L. M., Mitsumoto, H., Chung, W. K., Croft, G. F., Saphier, G., Leibel, R. L., Goland, R., et al. (2008) · Science · doi:10.1126/science.1158799 · Cited by 1,959 · see citing works

Amyotrophic Lateral Sclerosis

Rowland, L. P., & Shneider, N. A. (2001) · New England Journal of Medicine · doi:10.1056/nejm200105313442207 · Cited by 1,851 · see citing works

Directed differentiation of embryonic stem cells into motor neurons.

Wichterle, H., Lieberam, I., Porter, J. A., & Jessell, T. (2002) · Cell · doi:10.1016/s0092-8674(02)00835-8 · Cited by 1,792 · see citing works

Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia

Boillée, S., Yamanaka, K., Lobsiger, C. S., Copeland, N. G., Jenkins, N. A., Kassiotis, G., Kollias, G., & Cleveland, D. W. (2006) · Science · doi:10.1126/science.1123511 · Cited by 1,623 · see citing works

The presence of malfolded proteins in the endoplasmic reticulum signals the induction of glucose-regulated proteins

Kozutsumi, Y., Segal, M. S., Normington, K., Gething, M.-J. H., & Sambrook, J. (1988) · Nature · doi:10.1038/332462a0 · Cited by 1,322 · see citing works

Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons

Nagai, M., Re, D. B., Nagata, T., Chalazonitis, A., Jessell, T. M., Wichterle, H., & Przedborski, S. (2007) · Nature Neuroscience · doi:10.1038/nn1876 · Cited by 1,220 · see citing works

Calcium and apoptosis: ER-mitochondria Ca2+ transfer in the control of apoptosis

Pinton, P., Giorgi, C., Siviero, R., Zecchini, E., & Rizzuto, R. (2008) · Oncogene · doi:10.1038/onc.2008.308 · Cited by 1,145 · see citing works

Cell death induced by endoplasmic reticulum stress

Iurlaro, R., & Muñoz‐Pinedo, C. (2015) · FEBS Journal · doi:10.1111/febs.13598 · Cited by 1,067 · see citing works

Non–cell autonomous toxicity in neurodegenerative disorders: ALS and beyond

Ilieva, H., Polymenidou, M., & Cleveland, D. W. (2009) · Journal of Cell Biology · doi:10.1083/jcb.200908164 · Cited by 1,008 · see citing works

The role of the transcriptional regulator Ptf1a in converting intestinal to pancreatic progenitors

Kawaguchi, Y., Cooper, B., Gannon, M., Ray, M., MacDonald, R. J., & Wright, C. V. E. (2002) · Nature Genetics · doi:10.1038/ng959 · Cited by 1,007 · see citing works

ER stress and the unfolded protein response in neurodegeneration

Hetz, C. A., & Saxena, S. (2017) · Nature Reviews Neurology · doi:10.1038/nrneurol.2017.99 · Cited by 1,006 · see citing works

K-252 compounds, novel and potent inhibitors of protein kinase C and cyclic nucleotide-dependent protein kinases

Kase, H., Iwahashi, K., Nakanishi, S., Matsuda, Y., Yamada, K., Takahashi, M., Murakata, C., Sato, A., & Kaneko, M. (1987) · Biochemical and Biophysical Research Communications · doi:10.1016/0006-291x(87)90293-2 · Cited by 939 · see citing works

RNA Toxicity from the ALS/FTD C9ORF72 Expansion Is Mitigated by Antisense Intervention

Donnelly, C. J., Zhang, P.-W., Pham, J. T., Haeusler, A. R., Mistry, N. A., Vidensky, S., Daley, E. L., Poth, E. M., Hoover, B., Fines, D. M., et al. (2013) · Neuron · doi:10.1016/j.neuron.2013.10.015 · Cited by 877 · see citing works

Non–cell autonomous effect of glia on motor neurons in an embryonic stem cell–based ALS model

Di Giorgio, F. P., Carrasco, M. A., Siao, M. C., Maniatis, T., & Eggan, K. (2007) · Nature Neuroscience · doi:10.1038/nn1885 · Cited by 799 · see citing works

Astrocytes from familial and sporadic ALS patients are toxic to motor neurons

Haidet-Phillips, A. M., Hester, M. E., Miranda, C. J., Meyer, K., Braun, L., Frakes, A., Song, S., Likhite, S., Murtha, M. J., Foust, K. D., et al. (2011) · Nature Biotechnology · doi:10.1038/nbt.1957 · Cited by 782 · see citing works

Endoplasmic Reticulum Stress Sensing in the Unfolded Protein ResponsePreprint

Gardner, B. M., Pincus, D., Gotthardt, K., Gallagher, C. M., & Walter, P. (2013) · Cold Spring Harbor Perspectives in Biology · doi:10.1101/cshperspect.a013169 · Cited by 716 · see citing works

Rapid “Open-Source” Engineering of Customized Zinc-Finger Nucleases for Highly Efficient Gene Modification

Maeder, M. L., Thibodeau-Beganny, S., Osiak, A., Wright, D. A., Anthony, R. M., Eichtinger, M., Jiang, T., Foley, J. E., Winfrey, R. J., Townsend, J. A., et al. (2008) · Molecular Cell · doi:10.1016/j.molcel.2008.06.016 · Cited by 708 · see citing works

Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS

Bosco, D. A., Morfini, G. A., Karabacak, M., Song, Y., Gros‐Louis, F., Pasinelli, P., Goolsby, H., Fontaine, B. A., Lemay, N., McKenna-Yasek, D. M., et al. (2010) · Nature Neuroscience · doi:10.1038/nn.2660 · Cited by 685 · see citing works

Targeting RNA Foci in iPSC-Derived Motor Neurons from ALS Patients with a <i>C9ORF72</i> Repeat Expansion

Sareen, D., O’Rourke, J. G., Meera, P., Muhammad, A. K. M. G., Grant, S., Simpkinson, M., Bell, S., Carmona, S., Ornelas, L., Sahabian, A., et al. (2013) · Science Translational Medicine · doi:10.1126/scitranslmed.3007529 · Cited by 670 · see citing works

Axonal Transport of TDP-43 mRNA Granules Is Impaired by ALS-Causing Mutations

Alami, N. H., Smith, R. B., Carrasco, M. A., Williams, L. A., Winborn, C. S., Han, S. S. W., Kiskinis, E., Winborn, B., Freibaum, B. D., Kanagaraj, A., et al. (2014) · Neuron · doi:10.1016/j.neuron.2013.12.018 · Cited by 660 · see citing works

Intrinsic Membrane Hyperexcitability of Amyotrophic Lateral Sclerosis Patient-Derived Motor Neurons

Wainger, B. J., Kiskinis, E., Mellin, C., Wiskow, O., Han, S. S. W., Sandoe, J., Perez, N. P., Williams, L. A., Lee, S., Boulting, G. L., et al. (2014) · Cell Reports · doi:10.1016/j.celrep.2014.03.019 · Cited by 652 · see citing works

Abnormal excitatory amino acid metabolism in amyotrophic lateral sclerosis

Rothstein, J. D., Tsai, G. E., Kuncl, R. W., Clawson, L., Cornblath, D. R., Drachman, D. B., Pestronk, A., Stauch, B. L., & Coyle, J. T. (1990) · Annals of Neurology · doi:10.1002/ana.410280106 · Cited by 637 · see citing works

A role for motoneuron subtype–selective ER stress in disease manifestations of FALS mice

Saxena, S., Cabuy, E., & Caroni, P. (2009) · Nature Neuroscience · doi:10.1038/nn.2297 · Cited by 600 · see citing works

Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis

Kang, S. H., Li, Y., Fukaya, M., Lorenzini, I., Cleveland, D. W., Ostrow, L. W., Rothstein, J. D., & Bergles, D. E. (2013) · Nature Neuroscience · doi:10.1038/nn.3357 · Cited by 599 · see citing works

Novel genes associated with amyotrophic lateral sclerosis: diagnostic and clinical implications

Chia, R., Chiò, A., & Traynor, B. J. (2017) · The Lancet Neurology · doi:10.1016/s1474-4422(17)30401-5 · Cited by 576 · see citing works

Drug Screening for ALS Using Patient-Specific Induced Pluripotent Stem Cells

Egawa, N., Kitaoka, S., Tsukita, K., Naitoh, M., Takahashi, K., Yamamoto, T., Adachi, F., Kondo, T., Okita, K., Asaka, I., et al. (2012) · Science Translational Medicine · doi:10.1126/scitranslmed.3004052 · Cited by 552 · see citing works

Role of eIF2α Kinases in Translational Control and Adaptation to Cellular StressPreprint

Wek, R. C. (2018) · Cold Spring Harbor Perspectives in Biology · doi:10.1101/cshperspect.a032870 · Cited by 549 · see citing works

XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy

Hetz, C., Thielen, P., Matus, S., Nassif, M., Court, F., Kiffin, R., Martinez, G., Cuervo, A. M., Brown, R. H., & Glimcher, L. H. (2009) · Genes &amp; Development · doi:10.1101/gad.1830709 · Cited by 530 · see citing works

ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1Preprint

Nishitoh, H., Kadowaki, H., Nagai, A., Maruyama, T., Yokota, T., Fukutomi, H., Noguchi, T., Matsuzawa, A., Takeda, K., & Ichijo, H. (2008) · Genes & Development · doi:10.1101/gad.1640108 · Cited by 510 · see citing works

Human Embryonic Stem Cell-Derived Motor Neurons Are Sensitive to the Toxic Effect of Glial Cells Carrying an ALS-Causing Mutation

Giorgio, F. P. D., Boulting, G. L., Bobrowicz, S., & Eggan, K. C. (2008) · Cell stem cell · doi:10.1016/j.stem.2008.09.017 · Cited by 488 · see citing works

Motor Neuron Diversity in Development and Disease

Kanning, K. C., Kaplan, A., & Henderson, C. E. (2010) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.051508.135722 · Cited by 472 · see citing works

Pathways Disrupted in Human ALS Motor Neurons Identified through Genetic Correction of Mutant SOD1

Kiskinis, E., Sandoe, J., Williams, L. A., Boulting, G. L., Moccia, R., Wainger, B. J., Han, S., Peng, T., Thams, S., Mikkilineni, S., et al. (2014) · Cell stem cell · doi:10.1016/j.stem.2014.03.004 · Cited by 441 · see citing works

Combinatorial analysis of developmental cues efficiently converts human pluripotent stem cells into multiple neuronal subtypes

Maury, Y., Côme, J., Piskorowski, R. A., Salah‐Mohellibi, N., Chevaleyre, V., Peschanski, M., Martinat, C., & Nedelec, S. (2014) · Nature Biotechnology · doi:10.1038/nbt.3049 · Cited by 410 · see citing works

Paullones are potent inhibitors of glycogen synthase kinase‐3β and cyclin‐dependent kinase 5/p25

Leost, M., Schultz, C., Link, A., Wu, Y., Biernat, J., Mandelkow, E., Bibb, J. A., Snyder, G. L., Greengard, P., Zaharevitz, D. W., et al. (2000) · European Journal of Biochemistry · doi:10.1046/j.1432-1327.2000.01673.x · Cited by 378 · see citing works

ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function

Sharma, A., Lyashchenko, A. K., Lü, L., Nasrabady, S. E., Elmaleh, M., Mendelsohn, M. M., Nemes, A., Tapia, J. C., Mentis, G. Z., & Shneider, N. A. (2016) · Nature Communications · doi:10.1038/ncomms10465 · Cited by 339 · see citing works

Paullones, a Series of Cyclin-Dependent Kinase Inhibitors: Synthesis, Evaluation of CDK1/Cyclin B Inhibition, and in Vitro Antitumor Activity

Schultz, C., Link, A., Leost, M., Zaharevitz, D. W., Gussio, R., Sausville, E. A., Meijer, L., & Kunick, C. (1999) · Journal of Medicinal Chemistry · doi:10.1021/jm9900570 · Cited by 334 · see citing works

Motor neuron vulnerability and resistance in amyotrophic lateral sclerosis

Nijssen, J., Comley, L. H., & Hedlund, E. (2017) · Acta Neuropathologica · doi:10.1007/s00401-017-1708-8 · Cited by 334 · see citing works

Age-Dependent Penetrance of Disease in a Transgenic Mouse Model of Familial Amyotrophic Lateral Sclerosis

Chiu, A. Y., Zhai, P., Canto, M. C. D., Peters, T. M., Kwon, Y. W., Prattis, S. M., & Gurney, M. E. (1995) · Molecular and Cellular Neuroscience · doi:10.1006/mcne.1995.1027 · Cited by 326 · see citing works

VDAC1 selectively transfers apoptotic Ca2+ signals to mitochondria

Stefani, D. D., Bononi, A., Romagnoli, A., Messina, A. A., Pinto, V. D., Pinton, P., & Rizzuto, R. (2011) · Cell Death and Differentiation · doi:10.1038/cdd.2011.92 · Cited by 323 · see citing works

Emerging mechanisms of molecular pathology in ALS

Peters, O. M., Ghasemi, M., & Brown, R. H. (2015) · Journal of Clinical Investigation · doi:10.1172/jci71601 · Cited by 319 · see citing works

Neuronal Matrix Metalloproteinase-9 Is a Determinant of Selective Neurodegeneration

Kaplan, A., Spiller, K. J., Towne, C. L., Kanning, K. C., Choe, G. T., Geber, A. B., Akay, T., Aebischer, P., & Henderson, C. E. (2014) · Neuron · doi:10.1016/j.neuron.2013.12.009 · Cited by 309 · see citing works

Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease

Keene, C. D., Rodrigues, C. M. P., Eich, T., Chhabra, M. S., Steer, C. J., & Low, W. C. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.162362299 · Cited by 306 · see citing works

A Small Molecule Screen in Stem-Cell-Derived Motor Neurons Identifies a Kinase Inhibitor as a Candidate Therapeutic for ALS

Yang, Y. M., Gupta, S. K., Kim, K. J., Powers, B. E., Cerqueira, A., Wainger, B. J., Ngo, H. D., Rosowski, K. A., Schein, P. A., Ackeifi, C. A., et al. (2013) · Cell stem cell · doi:10.1016/j.stem.2013.04.003 · Cited by 305 · see citing works

Human iPSC-derived motoneurons harbouring TARDBP or C9ORF72 ALS mutations are dysfunctional despite maintaining viability

Devlin, A., Burr, K., Borooah, S., Foster, J. D., Cleary, E. M., Geti, I., Vallier, L., Shaw, C. E., Chandran, S., & Miles, G. B. (2015) · Nature Communications · doi:10.1038/ncomms6999 · Cited by 295 · see citing works

Neuroprotection through excitability and mTOR required in ALS motoneurons to delay disease and extend survival

Saxena, S., Roselli, F., Singh, K., Leptien, K., Julien, J.-P., Gros-Louis, F., & Caroni, P. (2013) · Neuron · doi:10.1016/j.neuron.2013.07.027 · Cited by 275 · see citing works

Interplay of endoplasmic reticulum stress and autophagy in neurodegenerative disorders

Cai, Y. C., Arikkath, J., Yang, L., Guo, M., Periyasamy, P., & Buch, S. J. (2016) · Autophagy · doi:10.1080/15548627.2015.1121360 · Cited by 258 · see citing works

T Cells Deficient in Inositol 1,4,5-Trisphosphate Receptor Are Resistant to Apoptosis

Jayaraman, T., & Marks, A. R. (1997) · Molecular and Cellular Biology · doi:10.1128/mcb.17.6.3005 · Cited by 256 · see citing works

Accelerated High-Yield Generation of Limb-Innervating Motor Neurons from Human Stem Cells

Amoroso, M. W., Croft, G. F., Williams, D. J., O’Keeffe, S., Carrasco, M. A., Davis, A. R., Roybon, L., Oakley, D. H., Maniatis, T., Henderson, C. E., et al. (2013) · Journal of Neuroscience · doi:10.1523/jneurosci.0906-12.2013 · Cited by 244 · see citing works

CRISPR–Cas9 screens in human cells and primary neurons identify modifiers of C9ORF72 dipeptide-repeat-protein toxicity

Kramer, N. J., Haney, M. S., Morgens, D. W., Jovičić, A., Couthouis, J., Li, A., Ousey, J., Ma, R., Bieri, G., Tsui, C. K., et al. (2018) · Nature Genetics · doi:10.1038/s41588-018-0070-7 · Cited by 226 · see citing works

Tauroursodeoxycholic acid in the treatment of patients with amyotrophic lateral sclerosis

Elia, A. E., Lalli, S., Monsurrò, M. R., Sagnelli, A., Taiello, A. C., Reggiori, B., Bella, V. L., Tedeschi, G., & Albanese, A. (2015) · European Journal of Neurology · doi:10.1111/ene.12664 · Cited by 225 · see citing works

Cyclic AMP Elevation Is Sufficient to Promote the Survival of Spinal Motor NeuronsIn Vitro

Hanson, M. G., Shen, S., Wiemelt, A. P., McMorris, F. A., & Barres, B. A. (1998) · Journal of Neuroscience · doi:10.1523/jneurosci.18-18-07361.1998 · Cited by 216 · see citing works

Functional Properties of Motoneurons Derived from Mouse Embryonic Stem Cells

Miles, G. B., Yohn, D. C., Wichterle, H., Jessell, T. M., Rafuse, V. F., & Brownstone, R. M. (2004) · Journal of Neuroscience · doi:10.1523/jneurosci.1972-04.2004 · Cited by 209 · see citing works

A Quantitative Framework to Evaluate Modeling of Cortical Development by Neural Stem Cells

Stein, J. L., de la Torre-Ubieta, L., Tian, Y., Parikshak, N. N., Hernández, I. A., Marchetto, M. C., Baker, D. K., Lu, D., Hinman, C. R., Lowe, J. K., et al. (2014) · Neuron · doi:10.1016/j.neuron.2014.05.035 · Cited by 201 · see citing works

Cyclopiazonic acid inhibition of the Ca2+-transport ATPase in rat skeletal muscle sarcoplasmic reticulum vesicles

Goeger, D. E., Riley, R. T., Dorner, J. W., & COLE, R. J. (1988) · Biochemical Pharmacology · doi:10.1016/0006-2952(88)90195-5 · Cited by 195 · see citing works

Translational profiling identifies a cascade of damage initiated in motor neurons and spreading to glia in mutant SOD1-mediated ALS

Sun, S., Sun, Y., Ling, S., Ferraiuolo, L., McAlonis‐Downes, M., Zou, Y., Drenner, K., Wang, Y., Ditsworth, D., Tokunaga, S., et al. (2015) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1520639112 · Cited by 194 · see citing works

TUDCA, a Bile Acid, Attenuates Amyloid Precursor Protein Processing and Amyloid-β Deposition in APP/PS1 Mice

Nunes, A. F., Amaral, J. D., Lo, A. C., Fonseca, M. B., Viana, R. J. S., Callaerts-Vegh, Z., D’Hooge, R., & Rodrigues, C. M. P. (2012) · Molecular Neurobiology · doi:10.1007/s12035-012-8256-y · Cited by 192 · see citing works

A follow-up study of blood pressure and cerebral white matter lesions

de Leeuw, F., de Groot, J. C., Oudkerk, M., Witteman, J. C. M., Albert Hofman, D., van Gijn, J., & Breteler, M. M. B. (1999) · Annals of Neurology · doi:10.1002/1531-8249(199912)46:6 · Cited by 192 · see citing works

Intracerebroventricular infusion of monoclonal antibody or its derived Fab fragment against misfolded forms of SOD1 mutant delays mortality in a mouse model of ALS

Gros‐Louis, F., Soucy, G., Larivière, R., & Julien, J. (2010) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2010.06683.x · Cited by 189 · see citing works

Ca2+, mitochondria and selective motoneuron vulnerability: implications for ALS

von Lewinski, F., & Keller, B. U. (2005) · Trends in Neurosciences · doi:10.1016/j.tins.2005.07.001 · Cited by 186 · see citing works

Protein Kinase Cθ Is Required for Autophagy in Response to Stress in the Endoplasmic Reticulum

Sakaki, K., Wu, J., & Kaufman, R. J. (2008) · Journal of Biological Chemistry · doi:10.1074/jbc.m710209200 · Cited by 179 · see citing works

Mitochondrial dysfunction and intracellular calcium dysregulation in ALS

Kawamata, H., & Manfredi, G. (2010) · Mechanisms of Ageing and Development · doi:10.1016/j.mad.2010.05.003 · Cited by 173 · see citing works

Tauroursodeoxycholic acid prevents MPTP-induced dopaminergic cell death in a mouse model of Parkinson's disease

Castro-Caldas, M., Carvalho, A. N., Rodrigues, E., Henderson, C. J., Wolf, C. R., Rodrigues, C. M. P., & Gama, M. J. (2012) · Molecular Neurobiology · doi:10.1007/s12035-012-8295-4 · Cited by 156 · see citing works

Direct Lineage Reprogramming Reveals Disease-Specific Phenotypes of Motor Neurons from Human ALS Patients

Liu, M.-L., Zang, T., & Zhang, C. (2015) · Cell Reports · doi:10.1016/j.celrep.2015.12.018 · Cited by 156 · see citing works

C9ORF72 interaction with cofilin modulates actin dynamics in motor neurons

Sivadasan, R., Hornburg, D., Drepper, C., Frank, N., Jablonka, S., Hansel, A., Lojewski, X., Sterneckert, J. L., Hermann, A., Shaw, P. J., et al. (2016) · Nature Neuroscience · doi:10.1038/nn.4407 · Cited by 148 · see citing works

Defining the nature of human pluripotent stem cell progeny

Patterson, M., Chan, D. N., Ha, I., Case, D., Cui, Y., Handel, B. V., Mikkola, H. K., & Lowry, W. E. (2012) · Cell Research · doi:10.1038/cr.2011.133 · Cited by 145 · see citing works

Progressive aggregation despite chaperone associations of a mutant SOD1-YFP in transgenic mice that develop ALS

Wang, J., Farr, G. W., Zeiss, C. J., Rodriguez‐Gil, D. J., Wilson, J. H., Furtak, K., Rutkowski, D. T., Kaufman, R. J., Ruse, C. I., Yates, J. R., et al. (2009) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0813045106 · Cited by 143 · see citing works

A Bile Acid Protects against Motor and Cognitive Deficits and Reduces Striatal Degeneration in the 3-Nitropropionic Acid Model of Huntington's Disease

Keene, C. D., Rodrigues, C. M. P., Eich, T., Linehan-Stieers, C., Abt, A., Kren, B. T., Steer, C. J., & Low, W. C. (2001) · Experimental Neurology · doi:10.1006/exnr.2001.7755 · Cited by 132 · see citing works

Protein Kinase C-independent Activation of Protein Kinase D Is Involved in BMP-2-induced Activation of Stress Mitogen-activated Protein Kinases JNK and p38 and Osteoblastic Cell Differentiation

Lemonnier, J., Ghayor, C., Guicheux, J., & Caverzasio, J. (2003) · Journal of Biological Chemistry · doi:10.1074/jbc.m308665200 · Cited by 132 · see citing works

Neuromuscular transmission in amyotrophic lateral sclerosis

Maselli, R. A., Wollman, R. L., Leung, C. G., Distad, B. J., Palombi, S., Richman, D. P., Salazar‐Grueso, E. F., & Roos, R. P. (1993) · Muscle & Nerve · doi:10.1002/mus.880161109 · Cited by 130 · see citing works

The assessment of diabetic polyneuropathy in daily clinical practice: Reproducibility and validity of Semmes Weinstein monofilaments examination and clinical neurological examination

Valk, G. D., de Sonnaville, J. J. J., van Houtum, W. H., Heine, R. J., van Eijk, J. T. M., Bouter, L. M., & Bertelsmann, F. W. (1997) · Muscle & Nerve · doi:10.1002/(sici)1097-4598(199701)20:1 · Cited by 128 · see citing works

Rbfox Splicing Factors Promote Neuronal Maturation and Axon Initial Segment Assembly

Jacko, M., Weyn‐Vanhentenryck, S. M., Smerdon, J. W., Yan, R., Feng, H., Williams, D. J., Pai, J. A., Xu, K., Wichterle, H., & Zhang, C. (2018) · Neuron · doi:10.1016/j.neuron.2018.01.020 · Cited by 126 · see citing works

4-Aminopyridine Induced Activity Rescues Hypoexcitable Motor Neurons from Amyotrophic Lateral Sclerosis Patient-Derived Induced Pluripotent Stem Cells

Naujock, M., Stanslowsky, N., Bufler, S., Naumann, M., Reinhardt, P., Sterneckert, J. L., Kefalakes, E., Kassebaum, C., Bursch, F., Lojewski, X., et al. (2016) · Stem Cells · doi:10.1002/stem.2354 · Cited by 125 · see citing works

Bile Acids in Neurodegenerative Disorders

Ackerman, H. D., & Gerhard, G. S. (2016) · Frontiers in Aging Neuroscience · doi:10.3389/fnagi.2016.00263 · Cited by 120 · see citing works

Impairment of mitochondrial calcium handling in a mtSOD1 cell culture model of motoneuron disease

Jaiswal, M. K., Zech, W., Goos, M., Leutbecher, C., Ferri, A., Zippelius, A., Carrı̀, M. T., Nau, R., & Keller, B. U. (2009) · BMC Neuroscience · doi:10.1186/1471-2202-10-64 · Cited by 111 · see citing works

K252a and CEP1347 Are Neuroprotective Compounds That Inhibit Mixed-lineage Kinase-3 and Induce Activation of Akt and ERK

Roux, P. P., Dorval, G., Boudreau, M., Angers‐Loustau, A., Morris, S. J., Makkerh, J., & Barker, P. A. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m203428200 · Cited by 103 · see citing works

Cu/Zn Superoxide Dismutase Typical for Familial Amyotrophic Lateral Sclerosis Increases the Vulnerability of Mitochondria and Perturbs Ca2+ Homeostasis in SOD1G93A Mice

Jaiswal, M. K., & Keller, B. U. (2008) · Molecular Pharmacology · doi:10.1124/mol.108.050831 · Cited by 98 · see citing works

Reduced Calreticulin Levels Link Endoplasmic Reticulum Stress and Fas-Triggered Cell Death in Motoneurons Vulnerable to ALS

Bernard‐Marissal, N., Moumen, A., Sunyach, C., Pellegrino, C., Dudley, K., Henderson, C. E., Raoul, C., & Pettmann, B. (2012) · Journal of Neuroscience · doi:10.1523/jneurosci.5431-11.2012 · Cited by 95 · see citing works

Chemical chaperone, TUDCA unlike PBA, mitigates protein aggregation efficiently and resists ER and non-ER stress induced HepG2 cell death

Uppala, J. K., Gani, A. R., & Ramaiah, K. V. A. (2017) · Scientific Reports · doi:10.1038/s41598-017-03940-1 · Cited by 90 · see citing works

Interferon-γ potentiates endoplasmic reticulum stress-induced death by reducing pancreatic beta cell defence mechanisms

Pirot, P., Eizirik, D. L., & Cardozo, A. K. (2006) · Diabetologia · doi:10.1007/s00125-006-0214-7 · Cited by 87 · see citing works

Emerging mechanisms of molecular pathology in ALS

Peters, O. M., Ghasemi, M., & Brown, R. H. (2015) · Journal of Clinical Investigation · doi:10.1172/jci82693 · Cited by 83 · see citing works

Calcium dysregulation, mitochondrial pathology and protein aggregation in a culture model of amyotrophic lateral sclerosis: Mechanistic relationship and differential sensitivity to intervention

Tradewell, M. L., Cooper, L. A., Minotti, S., & Durham, H. D. (2011) · Neurobiology of Disease · doi:10.1016/j.nbd.2011.01.016 · Cited by 81 · see citing works

Glycogen synthase kinase-3 regulates endoplasmic reticulum (ER) stress-induced CHOP expression in neuronal cells

Meares, G. P., Mines, M. A., Beurel, E., Eom, T.-Y., Song, L., Zmijewska, A. A., & Jope, R. S. (2011) · Experimental Cell Research · doi:10.1016/j.yexcr.2011.02.012 · Cited by 80 · see citing works

Discovery of Inhibitors of Microglial Neurotoxicity Acting Through Multiple Mechanisms Using a Stem-Cell-Based Phenotypic Assay

Höing, S., Rudhard, Y., Reinhardt, P., Glatza, M., Stehling, M., Wu, G., Peiker, C., Böcker, A., Parga, J. A., Bunk, E. C., et al. (2012) · Cell stem cell · doi:10.1016/j.stem.2012.07.005 · Cited by 77 · see citing works

The mitochondrial calcium regulator cyclophilin d is an essential component of oestrogen-mediated neuroprotection in amyotrophic lateral sclerosis

Kim, H. J., Magranè, J., Starkov, A. A., & Manfredi, G. (2012) · Brain · doi:10.1093/brain/aws208 · Cited by 64 · see citing works

Relation of neuronal endoplasmic reticulum calcium homeostasis to ribosomal aggregation and protein synthesis: implications for stress-induced suppression of protein synthesis

Doutheil, J., Gissel, C., Oschlies, U., Hossmann, K., & Paschen, W. (1997) · Brain Research · doi:10.1016/s0006-8993(97)00899-8 · Cited by 60 · see citing works

Direct and Indirect Roles of Cyclin-dependent Kinase 5 as an Upstream Regulator in the c-Jun NH2-Terminal Kinase Cascade: Relevance to Neurotoxic Insults in Alzheimer's Disease

Sun, K.-H., Lee, H., Smith, M. A., & Shah, K. (2009) · Molecular Biology of the Cell · doi:10.1091/mbc.e09-05-0433 · Cited by 59 · see citing works

Development of MAP4 Kinase Inhibitors as Motor Neuron-Protecting Agents

Bos, P. H., Lowry, E. R., Costa, J. A., Thams, S., Garcia-Diaz, A., Zask, A., Wichterle, H., & Stockwell, B. R. (2019) · Cell chemical biology · doi:10.1016/j.chembiol.2019.10.005 · Cited by 59 · see citing works

The motor end‐plate fine structure and ultrastructural localization of acetylcholine receptors in amyotrophic lateral sclerosis

Tsujihata, M., Hazama, R., Yoshimura, T., Satoh, A., Mori, M., & Nagataki, S. (1984) · Muscle & Nerve · doi:10.1002/mus.880070310 · Cited by 42 · see citing works

Stem cell-derived cranial and spinal motor neurons reveal proteostatic differences between ALS resistant and sensitive motor neurons

An, D., Fujiki, R., Iannitelli, D. E., Smerdon, J. W., Maity, S., Rose, M. F., Gelber, A., Wanaselja, E. K., Yagudayeva, I., Lee, J. Y., et al. (2019) · eLife · doi:10.7554/elife.44423 · Cited by 41 · see citing works

Intermediate filament protein accumulation in motor neurons derived from giant axonal neuropathy iPSCs rescued by restoration of gigaxonin.

Johnson-Kerner, B., Ahmad, F., Diaz, A. G., Greene, J. P., Gray, S., Samulski, R., Chung, W., Coster, R. V., Maertens, P., Noggle, S., et al. (2015) · Human Molecular Genetics · doi:10.1093/hmg/ddu556 · Cited by 38 · see citing works

Creatine-supplemented diet extends Purkinje cell survival in spinocerebellar ataxia type 1 transgenic mice but does not prevent the ataxic phenotype

Kaemmerer, W. F., Rodrigues, C. M. P., Steer, C. J., & Low, W. C. (2001) · Neuroscience · doi:10.1016/s0306-4522(01)00017-3 · Cited by 37 · see citing works

Subtype diversification and synaptic specificity of stem cell-derived spinal interneurons

Hoang, P. T., Chalif, J. I., Bikoff, J. B., Jessell, T. M., Mentis, G. Z., & Wichterle, H. (2018) · Neuron · doi:10.1016/j.neuron.2018.09.016 · Cited by 25 · see citing works

The indolocarbazole Gö6976 protects neurons from lipopolysaccharide/interferon-γ-induced cytotoxicity in murine neuron/glia co-cultures

Jeohn, G., Wilson, B. C., Wetsel, W. C., & Hong, J. (2000) · Molecular Brain Research · doi:10.1016/s0169-328x(00)00082-6 · Cited by 22 · see citing works

Microbiota–gut–brain axis and its therapeutic applications in neurodegenerative diseases

Loh, J. S., Mak, W. Q., Tan, L. K. S., Ng, C. X., Chan, H. H., Yeow, S. H., Foo, J. B., Ong, Y. S., How, C. W., & Khaw, K. Y. (2024) · Signal Transduction and Targeted Therapy · doi:10.1038/s41392-024-01743-1 · Cited by 1,193 · see citing works

Bile acid signaling in neurodegenerative and neurological disorders

Grant, S. M., & DeMorrow, S. (2020) · International Journal of Molecular Sciences · doi:10.3390/ijms21175982 · Cited by 185 · see citing works

Tauroursodeoxycholic acid: a potential therapeutic tool in neurodegenerative diseases

Khalaf, K., Tornese, P., Cocco, A., & Albanese, A. (2022) · Translational Neurodegeneration · doi:10.1186/s40035-022-00307-z · Cited by 150 · see citing works

Roles of bile acids signaling in neuromodulation under physiological and pathological conditions

Chen, X., Huang, X., Wang, D., Yu, D., Hou, S., Cui, H., & Song, L. (2023) · Cell & Bioscience · doi:10.1186/s13578-023-01053-z · Cited by 120 · see citing works

Bile acids and neurological disease

Hurley, M. J., Bates, R., Macnaughtan, J., & Schapira, A. H. V. (2022) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2022.108311 · Cited by 82 · see citing works

The Role of Mitochondrial Dysfunction and ER Stress in TDP-43 and C9ORF72 ALS

Dafinca, R., Barbagallo, P., & Talbot, K. (2021) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2021.653688 · Cited by 73 · see citing works

Nutritional and metabolic factors in amyotrophic lateral sclerosis

Ludolph, A. C., Dupuis, L., Kasarskis, E. J., Steyn, F. J., Ngo, S. T., & McDermott, C. J. (2023) · Nature Reviews Neurology · doi:10.1038/s41582-023-00845-8 · Cited by 70 · see citing works

Protein aggregation and therapeutic strategies in SOD1- and TDP-43- linked ALS

Tsekrekou, M., Giannakou, M., Papanikolopoulou, K., & Σκρέτας, Γ. (2024) · Frontiers in Molecular Biosciences · doi:10.3389/fmolb.2024.1383453 · Cited by 69 · see citing works

Development of MAP4 Kinase Inhibitors as Motor Neuron-Protecting Agents

Bos, P. H., Lowry, E. R., Costa, J. A., Thams, S., Garcia-Diaz, A., Zask, A., Wichterle, H., & Stockwell, B. R. (2019) · Cell chemical biology · doi:10.1016/j.chembiol.2019.10.005 · Cited by 59 · see citing works

Mir-17∼92 Confers Motor Neuron Subtype Differential Resistance to ALS-Associated Degeneration

Tung, Y.-T., Peng, K.-C., Chen, Y., Yen, Y.-P., Chang, M., Thams, S., & Chen, J. (2019) · Cell stem cell · doi:10.1016/j.stem.2019.04.016 · Cited by 54 · see citing works

Emerging Roles of Bile Acids and TGR5 in the Central Nervous System: Molecular Functions and Therapeutic Implications

Romero‐Ramírez, L., & Mey, J. (2024) · International Journal of Molecular Sciences · doi:10.3390/ijms25179279 · Cited by 49 · see citing works

Stem cell-derived cranial and spinal motor neurons reveal proteostatic differences between ALS resistant and sensitive motor neurons

An, D., Fujiki, R., Iannitelli, D. E., Smerdon, J. W., Maity, S., Rose, M. F., Gelber, A., Wanaselja, E. K., Yagudayeva, I., Lee, J. Y., et al. (2019) · eLife · doi:10.7554/elife.44423 · Cited by 41 · see citing works

Altered mitochondria-associated ER membrane (MAM) function shifts mitochondrial metabolism in amyotrophic lateral sclerosis (ALS)

Larrea, D., Tamucci, K. A., Kabra, K., VELASCO, K. R., Yun, T. D., Pera, M., Montesinos, J., Agrawal, R. R., Paradas, C., Smerdon, J. W., et al. (2025) · Nature Communications · doi:10.1038/s41467-024-51578-1 · Cited by 39 · see citing works

Liver’s influence on the brain through the action of bile acids

Yeo, X. Y., Tan, L. Y., Chae, W. R., Lee, D., Lee, Y., Wüestefeld, T., & Jung, S. (2023) · Frontiers in Neuroscience · doi:10.3389/fnins.2023.1123967 · Cited by 33 · see citing works

Subtype diversification and synaptic specificity of stem cell-derived spinal interneurons

Hoang, P. T., Chalif, J. I., Bikoff, J. B., Jessell, T. M., Mentis, G. Z., & Wichterle, H. (2018) · Neuron · doi:10.1016/j.neuron.2018.09.016 · Cited by 25 · see citing works

Emerging Roles of Bile Acids in Neuroinflammation

Butcher, E. L., & Arthur, S. (2025) · International Journal of Molecular Sciences · doi:10.3390/ijms262311301 · Cited by 34 · see citing works

Efficient Neuroprotective Rescue of Sacsin-Related Disease Phenotypes in Zebrafish

Naef, V., Marchese, M., Ogi, A., Fichi, G., Galatolo, D., Licitra, R., Doccini, S., Verri, T., Argenton, F., Morani, F., et al. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22168401 · Cited by 28 · see citing works

Tauroursodeoxycholic acid: a bile acid that may be used for the prevention and treatment of Alzheimer’s disease

Song, H., Liu, J., Wang, L., Hu, X., Li, J., Zhu, L., Pang, R., & Zhang, A. (2024) · Frontiers in Neuroscience · doi:10.3389/fnins.2024.1348844 · Cited by 26 · see citing works

Histamine beyond its effects on allergy: Potential therapeutic benefits for the treatment of Amyotrophic Lateral Sclerosis (ALS)

Volonté, C., Apolloni, S., & Sabatelli, M. (2019) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2019.06.006 · Cited by 26 · see citing works

Tauroursodeoxycholic acid in patients with amyotrophic lateral sclerosis: The TUDCA-ALS trial protocol

Albanese, A., Ludolph, A. C., McDermott, C. J., Corcia, P., Damme, P. V., van den Berg, L. H., Hardiman, O. M., Rinaldi, G., Vanacore, N., Dickie, B., et al. (2022) · Frontiers in Neurology · doi:10.3389/fneur.2022.1009113 · Cited by 24 · see citing works

c-Jun N-Terminal Kinase Promotes Stress Granule Assembly and Neurodegeneration in C9orf72-Mediated ALS and FTD

Sahana, T. G., Chase, K. J., Liu, F., Lloyd, T. E., Rossoll, W., & Zhang, K. (2023) · Journal of Neuroscience · doi:10.1523/jneurosci.1799-22.2023 · Cited by 20 · see citing works

In a search for efficient treatment for amyotrophic lateral sclerosis: Old drugs for new approaches

Kukharsky, M. S., Skvortsova, V. I., Бачурин, С. О., & Buchman, V. L. (2020) · Medicinal Research Reviews · doi:10.1002/med.21725 · Cited by 21 · see citing works

Standardized Reporter Systems for Purification and Imaging of Human Pluripotent Stem Cell-derived Motor Neurons and Other Cholinergic Cells

Garcia-Diaz, A., Efe, G., Kabra, K., Patel, A., Lowry, E. R., Shneider, N. A., Corneo, B., & Wichterle, H. (2020) · Neuroscience · doi:10.1016/j.neuroscience.2020.06.028 · Cited by 20 · see citing works

Analysis of proteome-wide degradation dynamics in ALS SOD1 iPSC-derived patient neurons reveals disrupted VCP homeostasis

Tsioras, K., Smith, K. C., Edassery, S. L., Garjani, M., Li, Y., Williams, C., McKenna, E. D., Guo, W., Wilen, A. P., Hark, T. J., et al. (2023) · Cell Reports · doi:10.1016/j.celrep.2023.113160 · Cited by 20 · see citing works

The motor neuron m6A repertoire governs neuronal homeostasis and FTO inhibition mitigates ALS symptom manifestation

Yen, Y.-P., Lung, T.-H., Liau, E. S., Wu, C., Huang, G., Hsu, F.-Y., Chang, M., Yang, Z.-D., Huang, C.-Y., Zheng, Z., et al. (2025) · Nature Communications · doi:10.1038/s41467-025-59117-2 · Cited by 16 · see citing works

Small molecules targeting different cellular pathologies for the treatment of amyotrophic lateral sclerosis

El‐Mansy, M. F., Reidl, C. T., Rahaman, M., Özdi̇nler, P. H., & Silverman, R. B. (2023) · Medicinal Research Reviews · doi:10.1002/med.21974 · Cited by 16 · see citing works

Bile Acids Induce Neurite Outgrowth in Nsc-34 Cells via TGR5 and a Distinct Transcriptional Profile

Ackerman, H. D., & Gerhard, G. S. (2023) · Pharmaceuticals · doi:10.3390/ph16020174 · Cited by 13 · see citing works

Harnessing the Potential of Human Pluripotent Stem Cell-Derived Motor Neurons for Drug Discovery in Amyotrophic Lateral Sclerosis: From the Clinic to the Laboratory and Back to the Patient

Lamas, N. J., & Roybon, L. (2021) · Frontiers in Drug Discovery · doi:10.3389/fddsv.2021.773424 · Cited by 12 · see citing works

The role of the gut microbiome in neuroinflammation and chemotherapy-induced peripheral neuropathy

Shatunova, S., Aktar, R., Peiris, M., Lee, J. Y. P., Vetter, I., & Starobova, H. (2024) · European Journal of Pharmacology · doi:10.1016/j.ejphar.2024.176818 · Cited by 14 · see citing works

Swift induction of human spinal lower motor neurons and robust ALS cell screening via single-cell imaging

Setsu, S., Morimoto, S., Nakamura, S., Ozawa, F., Utami, K. H., Nishiyama, A., Suzuki, N., Aoki, M., Takeshita, Y., Tomari, Y., et al. (2024) · Stem Cell Reports · doi:10.1016/j.stemcr.2024.11.007 · Cited by 12 · see citing works

Discovery of a Potent and Selective CDKL5/GSK3 Chemical Probe That Is Neuroprotective

Ong, H. W., Liang, Y., Richardson, W. A., Lowry, E. R., Wells, C. I., Chen, X., Silvestre, M., Dempster, K., Silvaroli, J. A., Smith, J. L., et al. (2023) · ACS Chemical Neuroscience · doi:10.1021/acschemneuro.3c00135 · Cited by 12 · see citing works

Precision genetic cellular models identify therapies protective against ER stress

Lebedeva, I. V., Wagner, M. V., Sahdeo, S., Lu, Y., Anyanwu‐Ofili, A., Harms, M. B., Wadia, J. S., Rajagopal, G., Boland, M. J., & Goldstein, D. B. (2021) · Cell Death and Disease · doi:10.1038/s41419-021-04045-4 · Cited by 12 · see citing works

Deep Learning Analysis on Images of iPSC-derived Motor Neurons Carrying fALS-genetics Reveals Disease-Relevant PhenotypesPreprint

Atmaramani, R., Dreossi, T., Ford, K., Gan, L., Mitchell, J. M., Tu, S., Velayutham, J., Zeng, H., Chickering, M., Soare, T., et al. (2024) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2024.01.04.574270 · Cited by 11 · see citing works

Screens in aging-relevant human ALS-motor neurons identify MAP4Ks as therapeutic targets for the disease

Liu, M., Ma, S., Tai, W., Zhong, X., Ni, H., Zou, Y., Wang, J., & Zhang, C. (2024) · Cell Death and Disease · doi:10.1038/s41419-023-06395-7 · Cited by 10 · see citing works

Analyzing the ER stress response in ALS patient derived motor neurons identifies druggable neuroprotective targets

Watts, M. E., Giadone, R. M., Ordureau, A., Holton, K. M., Harper, J. W., & Rubin, L. L. (2024) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2023.1327361 · Cited by 7 · see citing works

Fighting Amyotrophic Lateral Sclerosis by Protecting the Liver? A Prospective Cohort Study

Zhu, L., Li, Y., Yu, X., Chen, Y., Zhang, J., Pang, C., Xie, J., Gao, L., Du, L., Cao, W., et al. (2024) · Annals of Neurology · doi:10.1002/ana.27115 · Cited by 7 · see citing works

Tauro-Urso-Deoxycholic Acid Trials in Amyotrophic Lateral Sclerosis: What is Achieved and What to Expect

Giudice, M. L., Cocco, A., Reggiardo, G., Lalli, S., & Albanese, A. (2023) · Clinical Drug Investigation · doi:10.1007/s40261-023-01324-0 · Cited by 6 · see citing works

Stem cell-derived brainstem mouse astrocytes obtain a neurotoxic phenotype in vitro upon neuroinflammation

Lindblad, C., Neumann, S., Kolbeinsdóttir, S., Zachariadis, V., Thelin, E. P., Enge, M., Thams, S., Brundin, L., & Svensson, M. A. (2023) · Journal of Inflammation · doi:10.1186/s12950-023-00349-8 · Cited by 5 · see citing works

Microbiota–gut–brain axis and bile acids–driven neuromodulation

Dong, T., Zhang, T., Wang, H., Zhang, J., Abdullah, R., Sun, B., & Peng, G. (2025) · Neural Regeneration Research · doi:10.4103/nrr.nrr-d-25-00927 · Cited by 4 · see citing works

Alteration of serum bile acids in amyotrophic lateral sclerosis

Lee, I., Nandakumar, R., & Haeusler, R. A. (2024) · Lipids · doi:10.1002/lipd.12390 · Cited by 4 · see citing works

ALS sensitive spinal motor neurons enter a degenerative downward spiral of impaired splicing and proteostasisPreprint

Iannitelli, D. E., Tan, A., Nguyen, E., Babu, A., Elorza, S. D., Joseph, T., Zaaijer, S., An, D., Ward, M. E., & Mazzoni, E. O. (2022) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2022.03.26.485939 · Cited by 4 · see citing works

Altered MAM function shifts mitochondrial metabolism in SOD1-mutant models of ALSPreprint

Larrea, D., Tamucci, K. A., Kabra, K., VELASCO, K. R., Yun, T. D., Pera, M., Montesinos, J., Agrawal, R. R., Smerdon, J. W., Lowry, E. R., et al. (2022) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2022.09.22.508778 · Cited by 2 · see citing works

The “metabolic axis” of ALS : The role of body weight in disease pathogenesis

Dupuis, L., & Chiò, A. (2023) · Muscle & Nerve · doi:10.1002/mus.27784 · Cited by 2 · see citing works

Chemical screens in aging-relevant human motor neurons identify MAP4Ks as therapeutic targets for amyotrophic lateral sclerosisPreprint

Liu, M., Ma, S., Tai, W., Zhong, X., Ni, H., Zou, Y., wang, J., & Zhang, C. (2023) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2023.04.24.538014 · Cited by 2 · see citing works

Tauroursodeoxycholic acid (TUDCA) ameliorates age‐related skeletal muscle loss

Carvalho, M. U. W. B. D., Barssotti, L., dos Santos, L. M. B., Rosa, L. R. D. O., Gomes‐Marcondes, M. C. C., CARNEIRO, E. M., Barbosa, H. C. L., & Zangerolamo, L. (2026) · The Journal of Physiology · doi:10.1113/jp290683 · Cited by 2 · see citing works

Manufacturing of CAR-T Cells: The Assembly Line

Wang, X., & Rivière, I. (2022) · Cancer drug discovery and develogment/Cancer drug discovery and development · doi:10.1007/978-3-030-87849-8_8 · Cited by 1 · see citing works

A Potent and Selective CDKL5/GSK3 Chemical Probe is NeuroprotectivePreprint

Ong, H. W., Liang, Y., Richardson, W. A., Lowry, E. R., Wells, C. I., Chen, X., Silvestre, M., Dempster, K., Silvaroli, J. A., Smith, J. L., et al. (2023) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2023.02.09.527935 · Cited by 1 · see citing works

A deep-learning-based screening platform in aging-relevant human motor neurons to identify therapeutic compounds for amyotrophic lateral sclerosisPreprint

Jalnapurkar, B., Le, D., Shainin, W., Zarcone, R., Bhatnagar, R., Chin, J., Corbin, C. K., Curt, C., Hodge, B. A., Martinez, F., et al. (2023) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2023.11.21.568079 · Cited by 1 · see citing works

Bile acids induce neurite outgrowth in NSC-34 cells via TGR5 and a distinct transcriptional profilePreprint

Gerhard, G. S., & Ackerman, H. D. (2022) · Research Square · doi:10.21203/rs.3.rs-2326293/v1

Theme 9 Clinical Trials and Trial Design

(2024) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2024.2403306

The C9orf72 dipeptide repeat protein poly-glycine-alanine sequesters nucleoporins and disrupts nuclear membranes

Richter, K. (2022) · theses.fr (ABES) · doi:10.70675/d4b295c1zf862z434dz9956z7eaa90a7d4a7

A Small Molecule Screen in Stem-Cell-Derived Motor Neurons Identifies a Kinase Inhibitor as a Candidate Therapeutic for ALS

Yang, Y. M., Gupta, S. K., Kim, K. J., Powers, B. E., Cerqueira, A., Wainger, B. J., Ngo, H. D., Rosowski, K. A., Schein, P. A., Ackeifi, C. A., et al. (2013) · Cell stem cell · doi:10.1016/j.stem.2013.04.003 · Cited by 305 · see citing works

Non-Cell-Autonomous Effect of Human SOD1G37R Astrocytes on Motor Neurons Derived from Human Embryonic Stem Cells

Marchetto, M. C. N., Muotri, A. R., Mu, Y., Smith, A. M., Cezar, G. G., & Gage, F. H. (2008) · Cell Stem Cell · doi:10.1016/j.stem.2008.10.001 · Cited by 424 · see citing works

Modulating Proteostatic Stress in Human Motor Neurons to Identify Neuroprotective Targets

Watts, M. E. (2021) · Digital Access to Scholarship at Harvard (DASH) (Harvard University)

Stem cell-derived cranial and spinal motor neurons reveal proteostatic differences between ALS resistant and sensitive motor neurons

An, D., Fujiki, R., Iannitelli, D. E., Smerdon, J. W., Maity, S., Rose, M. F., Gelber, A., Wanaselja, E. K., Yagudayeva, I., Lee, J. Y., et al. (2019) · eLife · doi:10.7554/elife.44423 · Cited by 41 · see citing works

Drug Screening for ALS Using Patient-Specific Induced Pluripotent Stem Cells

Egawa, N., Kitaoka, S., Tsukita, K., Naitoh, M., Takahashi, K., Yamamoto, T., Adachi, F., Kondo, T., Okita, K., Asaka, I., et al. (2012) · Science Translational Medicine · doi:10.1126/scitranslmed.3004052 · Cited by 552 · see citing works