The contribution of C. elegans neurogenetics to understanding neurodegenerative diseases

References

• Ahn, B.-H., Rhim, H., Kim, S.Y., Sung, Y.-M., Lee, M.-Y., Choi, J.-Y., … Min, D.S. (2002). alpha-synuclein interacts with phospholipase D isozymes and inhibits pervanadate-induced phospholipase D activation in human embryonic kidney-293 cells. The Journal of Biological Chemistry, 277(14), 12334–12342. doi:10.1074/jbc.M110414200
   PubMed Web of Science ®Google Scholar
• Al-Chalabi, A., Jones, A., Troakes, C., King, A., Al-Sarraj, S., & Van Den Berg, L.H. (2012). The genetics and neuropathology of amyotrophic lateral sclerosis. Acta Neuropathologica, 124(3), 339–352. doi:10.1007/s00401-012-1022-4
   PubMed Web of Science ®Google Scholar
• Alexander, A.G., Marfil, V., & Li, C. (2014). Use of Caenorhabditis elegans as a model to study Alzheimer’s disease and other neurodegenerative diseases. Frontiers in Genetics, 5, 279. doi:10.3389/fgene.2014.00279
   PubMed Web of Science ®Google Scholar
• Alonso, A.D.C., Zaidi, T., Novak, M., Grundke-Iqbal, I., & Iqbal, K. (2001). Hyperphosphorylation induces self-assembly of tau into tangles of paired helical filaments/straight filaments. Proceedings of the National Academy of Sciences of the United States of America, 98(12), 6923–6928. doi:10.1073/pnas.121119298
   PubMed Web of Science ®Google Scholar
• Armenti, S.T., Lohmer, L.L., Sherwood, D.R., & Nance, J. (2014). Repurposing an endogenous degradation system for rapid and targeted depletion of C. elegans proteins. Development, 141(23), 4640–4647. doi:10.1242/dev.115048
   PubMed Web of Science ®Google Scholar
• Ash, P.E.A., Zhang, Y.-J., Roberts, C.M., Saldi, T., Hutter, H., Buratti, E., … Link, C.D. (2010). Neurotoxic effects of TDP-43 overexpression in C. elegans. Human Molecular Genetics, 19(16), 3206–3218. doi:10.1093/hmg/ddq230
   PubMed Web of Science ®Google Scholar
• Asikainen, S., Vartiainen, S., Lakso, M., Nass, R., & Wong, G. (2005). Selective sensitivity of Caenorhabditis elegans neurons to RNA interference. Neuroreport, 16(18), 1995–1999. doi:10.1097/00001756-200512190-00005
   PubMed Web of Science ®Google Scholar
• Balendra, R., & Isaacs, A.M. (2018). C9orf72-mediated ALS and FTD: Multiple pathways to disease. Nature Reviews Neurology, 14(9), 544–558. doi:10.1038/s41582-018-0047-2
   PubMed Web of Science ®Google Scholar
• Bandres-Ciga, S., Diez-Fairen, M., Kim, J.J., & Singleton, A.B. (2020). Genetics of Parkinson’s disease: An introspection of its journey towards precision medicine. Neurobiology of Disease, 137, 104782. doi:10.1016/j.nbd.2020.104782
   PubMed Web of Science ®Google Scholar
• Baskoylu, S.N., Chapkis, N., Unsal, B., Lins, J., Schuch, K., Simon, J., & Hart, A.C. (2019). Disrupted Autophagy and Neuronal Dysfunction in C. elegans Knock-in Models of FUS Amyotrophic Lateral Sclerosis. BioRxiv, 7, 799932. doi:10.1101/799932
   Google Scholar
• Baskoylu, S.N., Yersak, J., O’Hern, P., Grosser, S., Simon, J., Kim, S., … Hart, A.C. (2018). Single copy/knock-in models of ALS SOD1 in C. elegans suggest loss and gain of function have different contributions to cholinergic and glutamatergic neurodegeneration. PLoS Genetics, 14(10), e1007682. doi:10.1371/journal.pgen.1007682
   PubMed Web of Science ®Google Scholar
• Bates, E.A., Victor, M., Jones, A.K., Shi, Y., & Hart, A.C. (2006). Differential contributions of Caenorhabditis elegans histone deacetylases to huntingtin polyglutamine toxicity. Journal of Neuroscience, 26(10), 2830–2838. doi:10.1523/JNEUROSCI.3344-05.2006
   PubMed Web of Science ®Google Scholar
• Bates, G. (2003). Huntingtin aggregation and toxicity in Huntington’s disease. The Lancet, 361(9369), 1642–1644. doi:10.1016/S0140-6736(03)13304-1
   PubMed Web of Science ®Google Scholar
• Bauer, P.O., & Nukina, N. (2009). The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies. Journal of Neurochemistry, 110(6), 1737–1765. doi:10.1111/j.1471-4159.2009.06302.x
   PubMed Web of Science ®Google Scholar
• Bellani, S., Sousa, V.L., Ronzitti, G., Valtorta, F., Meldolesi, J., & Chieregatti, E. (2010). The regulation of synaptic function by alpha-synuclein. Communicative & Integrative Biology, 3(2), 106–109. doi:10.4161/cib.3.2.10964
   PubMedGoogle Scholar
• Bellenguez, C., Grenier-Boley, B., & Lambert, J.C. (2020). Genetics of Alzheimer’s disease: Where we are, and where we are going. Current Opinion in Neurobiology, 61, 40–48. doi:10.1016/j.conb.2019.11.024
   PubMed Web of Science ®Google Scholar
• Benbow, S.J., Strovas, T.J., Darvas, M., Saxton, A., & Kraemer, B.C. (2020). Synergistic toxicity between tau and amyloid drives neuronal dysfunction and neurodegeneration in transgenic C. elegans. Human Molecular Genetics, 29(3), 495–505. doi:10.1093/hmg/ddz319
   PubMed Web of Science ®Google Scholar
• Bieschke, J., Cohen, E., Murray, A., Dillin, A., & Kelly, J.W. (2009). A kinetic assessment of the C. elegans amyloid disaggregation activity enables uncoupling of disassembly and proteolysis. Protein Science: A Publication of the Protein Society, 18(11), 2231–2241. doi:10.1002/pro.234
   PubMed Web of Science ®Google Scholar
• Bramblett, G.T., Goedert, M., Jakes, R., Merrick, S.E., Trojanowski, J.Q., & Lee, V.M.Y. (1993). Abnormal tau phosphorylation at Ser396 in Alzheimer’s disease recapitulates development and contributes to reduced microtubule binding. Neuron, 10(6), 1089–1099. doi:10.1016/0896-6273(93)90057-X
   PubMed Web of Science ®Google Scholar
• Brandt, R., Gergou, A., Wacker, I., Fath, T., & Hutter, H. (2009). A Caenorhabditis elegans model of tau hyperphosphorylation: Induction of developmental defects by transgenic overexpression of Alzheimer’s disease-like modified tau. Neurobiology of Aging, 30(1), 22–33. doi:10.1016/j.neurobiolaging.2007.05.011
   PubMed Web of Science ®Google Scholar
• Brignull, H.R., Moore, F.E., Tang, S.J., & Morimoto, R.I. (2006). Polyglutamine proteins at the pathogenic threshold display neuron-specific aggregation in a pan-neuronal Caenorhabditis elegans model. The Journal of Neuroscience: The Official Journal of the Society for Neuroscience, 26(29), 7597–7606. doi:10.1523/JNEUROSCI.0990-06.2006
   PubMed Web of Science ®Google Scholar
• Cabin, D.E., Shimazu, K., Murphy, D., Cole, N.B., Gottschalk, W., McIlwain, K.L., … Nussbaum, R.L. (2002). Synaptic vesicle depletion correlates with attenuated synaptic responses to prolonged repetitive stimulation in mice lacking α-synuclein. The Journal of Neuroscience: The Official Journal of the Society for Neuroscience, 22(20), 8797–8807. doi:10.1523/JNEUROSCI.22-20-08797.2002
   PubMed Web of Science ®Google Scholar
• Cabreiro, F., Ackerman, D., Doonan, R., Araiz, C., Back, P., Papp, D., … Gems, D. (2011). Increased life span from overexpression of superoxide dismutase in Caenorhabditis elegans is not caused by decreased oxidative damage. Free Radical Biology & Medicine, 51(8), 1575–1582. doi:10.1016/j.freeradbiomed.2011.07.020
   PubMed Web of Science ®Google Scholar
• Calixto, A., Chelur, D., Topalidou, I., Chen, X., & Chalfie, M. (2010). Enhanced neuronal RNAi in C. elegans using SID-1. Nature Methods, 7(7), 554–559. doi:10.1038/nmeth.1463
   PubMed Web of Science ®Google Scholar
• Canet-Avilés, R.M., Wilson, M.A., Miller, D.W., Ahmad, R., McLendon, C., Bandyopadhyay, S., … Cookson, M.R. (2004). The Parkinson’s disease protein DJ-1 is neuroprotective due to cysteine-sulfinic acid-driven mitochondrial localization. Proceedings of the National Academy of Sciences of the United States of America, 101(24), 9103–9108. doi:10.1073/pnas.0402959101
   PubMed Web of Science ®Google Scholar
• Cao, S., Gelwix, C.C., Caldwell, K.A., & Caldwell, G.A. (2005). Torsin-mediated protection from cellular stress in the dopaminergic neurons of Caenorhabditis elegans. The Journal of Neuroscience: The Official Journal of the Society for Neuroscience, 25(15), 3801–3812. doi:10.1523/JNEUROSCI.5157-04.2005
   PubMed Web of Science ®Google Scholar
• Chalfie, M., Tu, Y., Euskirchen, G., Ward, W.W., & Prasher, D.C. (1994). Green fluorescent protein as a marker for gene expression. Science, 263(5148), 802–805. doi:10.1126/science.8303295
   PubMed Web of Science ®Google Scholar
• Chen, C., Turnbull, D.M., & Reeve, A.K. (2019). Mitochondrial dysfunction in Parkinson’s disease—cause or consequence? Biology, 8(2), 38. doi:10.3390/biology8020038
   PubMed Web of Science ®Google Scholar
• Chen, C.H., Chen, Y.C., Jiang, H.C., Chen, C.K., & Pan, C.L. (2013). Neuronal aging: Learning from C. elegans. Journal of Molecular Signaling, 8 (1), 10–14. doi:10.1186/1750-2187-8-14
   PubMedGoogle Scholar
• Chew, Y.L., Fan, X., Götz, J., & Nicholas, H.R. (2013). PTL-1 regulates neuronal integrity and lifespan in C. elegans. Journal of Cell Science, 126(9), 2079–2091. doi:10.1242/jcs.jcs124404
   PubMed Web of Science ®Google Scholar
• Choudhary, B., Kamak, M., Ratnakaran, N., Kumar, J., Awasthi, A., Li, C., … Koushika, S.P. (2017). UNC-16/JIP3 regulates early events in synaptic vesicle protein trafficking via LRK18 1/LRRK2 and AP complexes. PLoS Genetics, 13(11), 1–25. doi:10.1371/journal.pgen.1007100
   Web of Science ®Google Scholar
• Cook, S.J., Jarrell, T.A., Brittin, C.A., Wang, Y., Bloniarz, A.E., Yakovlev, M.A., … Emmons, S.W. (2019). Whole-animal connectomes of both Caenorhabditis elegans sexes. Nature, 571(7763), 63–71. doi:10.1038/s41586-019-1352-7
   PubMed Web of Science ®Google Scholar
• Cooper, A.A., Gitler, A.D., Cashikar, A., Haynes, C.M., Hill, K.J., Bhullar, B., … Lindquist, S. (2006). Alpha-synuclein blocks ER-golgi traffic and Rab1 rescues neuron loss in Parkinson’s models. Science, 313(5785), 324–329. doi:10.1126/science.1129462
   PubMed Web of Science ®Google Scholar
• Cooper, J.F., Machiela, E., Dues, D.J., Spielbauer, K.K., Senchuk, M.M., & Van Raamsdonk, J.M. (2017). Activation of the mitochondrial unfolded protein response promotes longevity and dopamine neuron survival in Parkinson’s disease models. Scientific Reports, 7(1), 16441. doi:10.1038/s41598-017-16637-2
   PubMed Web of Science ®Google Scholar
• Cooper, J.F., & Van Raamsdonk, J.M. (2018). Modeling Parkinson’s disease in C. elegans. Journal of Parkinson’s Disease, 8(1), 17–32. doi:10.3233/JPD-171258
   PubMedGoogle Scholar
• Corrionero, A., & Horvitz, H.R. (2018). A C9orf72 ALS/FTD ortholog acts in endolysosomal degradation and lysosomal homeostasis. Current Biology, 28(10), 1522.e5–1535.e5. doi:10.1016/j.cub.2018.03.063
   Web of Science ®Google Scholar
• Cudkowicz, M.E., McKenna-Yasek, D., Sapp, P.E., Chin, W., Geller, B., Hayden, D.L., … Brown, R.H. (1997). Epidemiology of mutations in superoxide dismutase in amyotrophic lateral sclerosis. Annals of Neurology, 41(2), 210–221. doi:10.1002/ana.410410212
   PubMed Web of Science ®Google Scholar
• DeJesus-Hernandez, M., Mackenzie, I.R., Boeve, B.F., Boxer, A.L., Baker, M., Rutherford, N.J., … Rademakers, R. (2011). Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron, 72(2), 245–256. doi:10.1016/j.neuron.2011.09.011
   PubMed Web of Science ®Google Scholar
• Dickinson, D.J., Ward, J.D., Reiner, D.J., & Goldstein, B. (2013). Engineering the Caenorhabditis elegans genome using Cas9-triggered homologous recombination. Nature Methods, 10(10), 1028–1034. doi:10.1038/nmeth.2641
   PubMed Web of Science ®Google Scholar
• DiFiglia, M., Sapp, E., Chase, K.O., Davies, S.W., Bates, G.P., Vonsattel, J.P., & Aronin, N. (1997). Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science, 277(5334), 1990–1993. doi:10.1126/science.277.5334.1990
   PubMed Web of Science ®Google Scholar
• Dourlen, P., Kilinc, D., Malmanche, N., Chapuis, J., & Lambert, J.C. (2019). The new genetic landscape of Alzheimer’s disease: From amyloid cascade to genetically driven synaptic failure hypothesis? Acta Neuropathologica, 138(2), 221–236. doi:10.1007/s00401-019-02004-0
   PubMed Web of Science ®Google Scholar
• Estes, P.S., Boehringer, A., Zwick, R., Tang, J.E., Grigsby, B., & Zarnescu, D.C. (2011). Wild-type and A315T mutant TDP-43 exert differential neurotoxicity in a Drosophila model of ALS. Human Molecular Genetics, 20(12), 2308–2321. doi:10.1093/hmg/ddr124
   PubMed Web of Science ®Google Scholar
• Faber, P.W., Voisine, C., King, D.C., Bates, E.A., & Hart, A.C. (2002). Glutamine/proline-rich PQE-1 proteins protect Caenorhabditis elegans neurons from huntingtin polyglutamine neurotoxicity. Proceedings of the National Academy of Sciences of the United States of America, 99(26), 17131–17136. doi:10.1073/pnas.262544899
   PubMed Web of Science ®Google Scholar
• Fahn, S., & Sulzer, D. (2004). Neurodegeneration and neuroprotection in Parkinson disease. NeuroRx: The Journal of the American Society for Experimental Neurotherapeutics, 1(1), 139–154. doi:10.1602/neurorx.1.1.139
   PubMedGoogle Scholar
• Fan, H.-C., Ho, L.-I., Chi, C.-S., Chen, S.-J., Peng, G.-S., Chan, T.-M., … Harn, H.-J. (2014). Polyglutamine (PolyQ) diseases: Genetics to treatments. Cell Transplantation, 23(4–5), 441–458. doi:10.3727/096368914X678454
   PubMed Web of Science ®Google Scholar
• Fay, D.S., Fluet, A., Johnson, C.J., & Link, C.D. (1998). In vivo aggregation of beta-amyloid peptide variants. Journal of Neurochemistry, 71(4), 1616–1625. doi:10.1046/j.1471-4159.1998.71041616.x
   PubMed Web of Science ®Google Scholar
• Fire, A., Xu, S., Montgomery, M.K., Kostas, S.A., Driver, S.E., & Mello, C.C. (1998). Potent and specific genetic interference by double-stranded RNA in Caenorhabditis elegans. Nature, 391(6669), 806–811. doi:10.1038/35888
   PubMed Web of Science ®Google Scholar
• Florez-McClure, M.L., Hohsfield, L.A., Fonte, G., Bealor, M.T., & Link, C.D. (2007). Decreased insulin-receptor signaling promotes the autophagic degradation of beta-amyloid peptide in C. elegans. Autophagy, 3(6), 569–580. doi:10.4161/auto.4776
   PubMed Web of Science ®Google Scholar
• Fong, S., Teo, E., Ng, L.F., Chen, C.-B., Lakshmanan, L.N., Tsoi, S.Y., … Gruber, J. (2016). Energy crisis precedes global metabolic failure in a novel Caenorhabditis elegans Alzheimer disease model. Scientific Reports, 6(1), 33781. doi:10.1038/srep33781
   PubMed Web of Science ®Google Scholar
• Fonte, V., Kapulkin, W.J., Kapulkin, V., Taft, A., Fluet, A., Friedman, D., & Link, C.D. (2002). Interaction of intracellular beta amyloid peptide with chaperone proteins. Proceedings of the National Academy of Sciences of the United States of America, 99(14), 9439–9444. doi:10.1073/pnas.152313999
   PubMed Web of Science ®Google Scholar
• Friedland, A.E., Tzur, Y.B., Esvelt, K.M., Colaiácovo, M.P., Church, G.M., & Calarco, J.A. (2013). Heritable genome editing in C. elegans via a CRISPR-Cas9 system. Nature Methods, 10(8), 741–743. doi:10.1038/nmeth.2532
   PubMed Web of Science ®Google Scholar
• Fukuzono, T., Pastuhov, S.I., Fukushima, O., Li, C., Hattori, A., Iemura, S-i., … Hisamoto, N. (2016). Chaperone complex BAG2-HSC70 regulates localization of Caenorhabditis elegans leucine-rich repeat kinase LRK-1 to the Golgi. Genes to Cells: Devoted to Molecular & Cellular Mechanisms, 21(4), 311–324. doi:10.1111/gtc.12338
   PubMed Web of Science ®Google Scholar
• Gidalevitz, T., Ben-Zvi, A., Ho, K.H., Brignull, H.R., & Morimoto, R.I. (2006). Progressive disruption of cellular protein folding in models of polyglutamine diseases. Science, 311(5766), 1471–1474. doi:10.1126/science.1124514
   PubMed Web of Science ®Google Scholar
• Gidalevitz, T., Krupinski, T., Garcia, S., & Morimoto, R.I. (2009). Destabilizing protein polymorphisms in the genetic background direct phenotypic expression of mutant SOD1 toxicity. PLoS Genetics, 5(3), e1000399. doi:10.1371/journal.pgen.1000399
   PubMed Web of Science ®Google Scholar
• Giguère, N., Burke Nanni, S., & Trudeau, L.-E. (2018). On cell loss and selective vulnerability of neuronal populations in Parkinson’s disease. Frontiers in Neurology, 9, 455. doi:10.3389/fneur.2018.00455
   PubMed Web of Science ®Google Scholar
• Gitler, A.D., Chesi, A., Geddie, M.L., Strathearn, K.E., Hamamichi, S., Hill, K.J., … Lindquist, S. (2009). Alpha-synuclein is part of a diverse and highly conserved interaction network that includes PARK9 and manganese toxicity. Nature Genetics, 41(3), 308–315. doi:10.1038/ng.300
   PubMed Web of Science ®Google Scholar
• Goedert, M., Spillantini, M.G., Jakes, R., Rutherford, D., & Crowther, R.A. (1989). Multiple isoforms of human microtubule-associated protein tau: Sequences and localization in neurofibrillary tangles of Alzheimer’s disease. Neuron, 3(4), 519–526. doi:10.1016/0896-6273(89)90210-9
   PubMed Web of Science ®Google Scholar
• Gordon, P., Hingula, L., Krasny, M.L., Swienckowski, J.L., Pokrywka, N.J., & Raley-Susman, K.M. (2008). The invertebrate microtubule-associated protein PTL-1 functions in mechanosensation and development in Caenorhabditis elegans. Development Genes and Evolution, 218(10), 541–551. doi:10.1007/s00427-008-0250-z
   PubMed Web of Science ®Google Scholar
• Graffmo, K.S., Forsberg, K., Bergh, J., Birve, A., Zetterström, P., Andersen, P.M., … Brännström, T. (2013). Expression of wild-type human superoxide dismutase-1 in mice causes amyotrophic lateral sclerosis. Human Molecular Genetics, 22(1), 51–60. doi:10.1093/hmg/dds399
   PubMed Web of Science ®Google Scholar
• Green, D.R., & Llambi, F. (2015). Cell death signaling. Cold Spring Harbor Perspectives in Biology, 7(12), a006080. doi:10.1101/cshperspect.a006080
   PubMed Web of Science ®Google Scholar
• Hardy, J.A., & Higgins, G.A. (1992). Alzheimer’s disease: The amyloid cascade hypothesis. Science, 256(5054), 184–185. doi:10.1126/science.1566067
   PubMed Web of Science ®Google Scholar
• Hartman, J.H., Gonzalez-Hunt, C., Hall, S.M., Ryde, I.T., Caldwell, K.A., Caldwell, G.A., & Meyer, J.N. (2019). Genetic defects in mitochondrial dynamics in Caenorhabditis elegans impact ultraviolet C radiation- and 6-hydroxydopamine-induced neurodegeneration. International Journal of Molecular Sciences, 20(13), 3202. doi:10.3390/ijms20133202
   PubMed Web of Science ®Google Scholar
• Hassan, W.M., Merin, D.A., Fonte, V., & Link, C.D. (2009). AIP-1 ameliorates beta-amyloid peptide toxicity in a Caenorhabditis elegans Alzheimer’s disease model. Human Molecular Genetics, 18(15), 2739–2747. doi:10.1093/hmg/ddp209
   PubMed Web of Science ®Google Scholar
• Hengartner, M.O., & Horvitz, R.H. (1994). Programmed cell death in Caenorhabditis elegans. Current Opinion in Genetics & Development, 4(4), 581–586. doi:10.1016/0959-437X(94)90076-F
   PubMed Web of Science ®Google Scholar
• Hergesheimer, R.C., Chami, A.A., de Assis, D.R., Vourc’h, P., Andres, C.R., Corcia, P., … Blasco, H. (2019). The debated toxic role of aggregated TDP-43 in amyotrophic lateral sclerosis: A resolution in sight? Brain: A Journal of Neurology, 142(5), 1176–1194. doi:10.1093/brain/awz078
   PubMed Web of Science ®Google Scholar
• Hermann, A., Liewald, J.F., & Gottschalk, A. (2015). A photosensitive degron enables acute light-induced protein degradation in the nervous system. Current Biology , 25(17), R749–R750. doi:10.1016/j.cub.2015.07.040
   PubMed Web of Science ®Google Scholar
• Holdorff, B., Rodrigues e Silva, A.M., & Dodel, R. (2013). Centenary of Lewy bodies (1912-2012). Journal of Neural Transmission, 120(4), 509–516. doi:10.1007/s00702-013-0984-2
   PubMed Web of Science ®Google Scholar
• Hornsten, A., Lieberthal, J., Fadia, S., Malins, R., Ha, L., Xu, X., … Li, C. (2007). APL-1, a Caenorhabditis elegans protein related to the human beta-amyloid precursor protein, is essential for viability. Proceedings of the National Academy of Sciences of the United States of America, 104(6), 1971–1976. doi:10.1073/pnas.0603997104
   PubMed Web of Science ®Google Scholar
• Huang, M., Wang, B., Li, X., Fu, C., Wang, C., & Kang, X. (2019). Α-synuclein: A multifunctional player in exocytosis, endocytosis, and vesicle recycling. Frontiers in Neuroscience, 13, 28–28. doi:10.3389/fnins.2019.00028
   PubMed Web of Science ®Google Scholar
• Ikenaka, K., Kawai, K., Katsuno, M., Huang, Z., Jiang, Y.-M., Iguchi, Y., … Sobue, G. (2013). dnc-1/dynactin 1 knockdown disrupts transport of autophagosomes and induces motor neuron degeneration. PLoS One, 8(2), e54511. doi:10.1371/journal.pone.0054511
   PubMed Web of Science ®Google Scholar
• Ikenaka, K., Tsukada, Y., Giles, A.C., Arai, T., Nakadera, Y., Nakano, S., … Mori, I. (2019). A behavior-based drug screening system using a Caenorhabditis elegans model of motor neuron disease. Scientific Reports, 9(1), 1–10. doi:10.1038/s41598-019-46642-6
   PubMed Web of Science ®Google Scholar
• Janssen, J.C., Beck, J.A., Campbell, T.A., Dickinson, A., Fox, N.C., Harvey, R.J., … Collinge, J. (2003). Early onset familial Alzheimer’s disease: Mutation frequency in 31 families. Neurology, 60(2), 235–239. doi:10.1212/01.WNL.0000042088.22694.E3
   PubMed Web of Science ®Google Scholar
• Jeong, H., Then, F., Melia, T.J., Mazzulli, J.R., Cui, L., Savas, J.N., … Krainc, D. (2009). Acetylation targets mutant huntingtin to autophagosomes for degradation. Cell, 137(1), 60–72. doi:10.1016/j.cell.2009.03.018
   PubMed Web of Science ®Google Scholar
• Jiang, Y.-M., Yamamoto, M., Kobayashi, Y., Yoshihara, T., Liang, Y., Terao, S., … Sobue, G. (2005). Gene expression profile of spinal motor neurons in sporadic amyotrophic lateral sclerosis. Annals of Neurology, 57(2), 236–251. doi:10.1002/ana.20379
   PubMed Web of Science ®Google Scholar
• Jiang, Y.-M., Yamamoto, M., Tanaka, F., Ishigaki, S., Katsuno, M., Adachi, H., … Sobue, G. (2007). Gene expressions specifically detected in motor neurons (dynactin 1, early growth response 3, acetyl-CoA transporter, death receptor 5, and cyclin C) differentially correlate to pathologic markers in sporadic amyotrophic lateral sclerosis. Journal of Neuropathology and Experimental Neurology, 66(7), 617–627. doi:10.1097/nen.0b013e318093ece3
   PubMed Web of Science ®Google Scholar
• Joyce, P.I., Mcgoldrick, P., Saccon, R.A., Weber, W., Fratta, P., West, S.J., … Acevedo-Arozena, A. (2015). A novel SOD1-ALS mutation separates central and peripheral effects of mutant SOD1 toxicity. Human Molecular Genetics, 24(7), 1883–1897. doi:10.1093/hmg/ddu605
   PubMed Web of Science ®Google Scholar
• Kagan, B.L., Jang, H., Capone, R., Teran Arce, F., Ramachandran, S., Lal, R., & Nussinov, R. (2012). Antimicrobial properties of amyloid peptides. Molecular Pharmaceutics, 9(4), 708–717. doi:10.1021/mp200419b
   PubMed Web of Science ®Google Scholar
• Kamath, R.S., Fraser, A.G., Dong, Y., Poulin, G., Durbin, R., Gotta, M., … Ahringer, J. (2003). Systematic functional analysis of the Caenorhabditis elegans genome using RNAi. Nature, 421(6920), 231–237. doi:10.1038/nature01278
   PubMed Web of Science ®Google Scholar
• Kang, J., Lemaire, H.G., Unterbeck, A., Salbaum, J.M., Masters, C.L., Grzeschik, K.H., … Müller-Hill, B. (1987). The precursor of Alzheimer’s disease amyloid A4 protein resembles a cell-surface receptor. Nature, 325(6106), 733–736. doi:10.1038/325733a0
   PubMed Web of Science ®Google Scholar
• Karran, E., & De Strooper, B. (2016). The amyloid cascade hypothesis: Are we poised for success or failure? Journal of Neurochemistry, 139, 237–252. doi:10.1111/jnc.13632
   PubMed Web of Science ®Google Scholar
• Kaur, S.J., McKeown, S.R., & Rashid, S. (2016). Mutant SOD1 mediated pathogenesis of amyotrophic lateral sclerosis. Gene, 577(2), 109–118. doi:10.1016/j.gene.2015.11.049
   PubMed Web of Science ®Google Scholar
• Kidd, M. (1964). Alzheimer’s disease-an electron microscopical study. Brain: a Journal of Neurology, 87, 307–320. doi:10.1093/brain/87.2.307
   PubMed Web of Science ®Google Scholar
• Kim, W., Underwood, R.S., Greenwald, I., & Shaye, D.D. (2018). Ortholist 2: A new comparative genomic analysis of human and Caenorhabditis elegans genes. Genetics, 210(2), 445–461. doi:10.1534/genetics.118.301307
   PubMed Web of Science ®Google Scholar
• Koushika, S.P., Schaefer, A.M., Vincent, R., Willis, J.H., Bowerman, B., & Nonet, M.L. (2004). Mutations in Caenorhabditis elegans cytoplasmic dynein components reveal specificity of neuronal retrograde cargo. The Journal of Neuroscience: The Official Journal of the Society for Neuroscience, 24(16), 3907–3916. doi:10.1523/JNEUROSCI.5039-03.2004
   PubMed Web of Science ®Google Scholar
• Kraemer, B.C., & Schellenberg, G.D. (2007). SUT-1 enables tau-induced neurotoxicity in C. elegans. Human Molecular Genetics, 16(16), 1959–1971. doi:10.1093/hmg/ddm143
   PubMed Web of Science ®Google Scholar
• Kraemer, B.C., Zhang, B., Leverenz, J.B., Thomas, J.H., Trojanowski, J.Q., & Schellenberg, G.D. (2003). Neurodegeneration and defective neurotransmission in a Caenorhabditis elegans model of tauopathy. Proceedings of the National Academy of Sciences of the United States of America, 100(17), 9980–9985. doi:10.1073/pnas.1533448100
   PubMed Web of Science ®Google Scholar
• Kumar, A., Kumar, V., Singh, K., Kumar, S., Kim, Y.-S., Lee, Y.-M., & Kim, J.-J. (2020). Therapeutic advances for Huntington’s disease. Brain Sciences, 10(1), 43. doi:10.3390/brainsci10010043
   PubMed Web of Science ®Google Scholar
• Kuwahara, T., Koyama, A., Gengyo-Ando, K., Masuda, M., Kowa, H., Tsunoda, M., … Iwatsubo, T. (2006). Familial Parkinson mutant alpha-synuclein causes dopamine neuron dysfunction in transgenic Caenorhabditis elegans. The Journal of Biological Chemistry, 281(1), 334–340. doi:10.1074/jbc.M504860200
   PubMed Web of Science ®Google Scholar
• Kuwahara, T., Koyama, A., Koyama, S., Yoshina, S., Ren, C.-H., Kato, T., … Iwatsubo, T. (2008). A systematic RNAi screen reveals involvement of endocytic pathway in neuronal dysfunction in alpha-synuclein transgenic C. elegans. Human Molecular Genetics, 17(19), 2997–3009. doi:10.1093/hmg/ddn198
   PubMed Web of Science ®Google Scholar
• Kwiatkowski, T.J., Bosco, D.A., Leclerc, A.L., Tamrazian, E., Vanderburg, C.R., Russ, C., … Brown, R.H. (2009). Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science, 323(5918), 1205–1208. doi:10.1126/science.1166066
   PubMed Web of Science ®Google Scholar
• Lakso, M., Vartiainen, S., Moilanen, A.-M., Sirviö, J., Thomas, J.H., Nass, R., … Wong, G. (2003). Dopaminergic neuronal loss and motor deficits in Caenorhabditis elegans overexpressing human alpha-synuclein. Journal of Neurochemistry, 86(1), 165–172. doi:10.1046/j.1471-4159.2003.01809.x
   PubMed Web of Science ®Google Scholar
• Lee, J-y., Song, J., Kwon, K., Jang, S., Kim, C., Baek, K., … Park, C. (2012). Human DJ-1 and its homologs are novel glyoxalases. Human Molecular Genetics, 21(14), 3215–3225. doi:10.1093/hmg/dds155
   PubMed Web of Science ®Google Scholar
• Levitan, D., Doyle, T.G., Brousseau, D., Lee, M.K., Thinakaran, G., Slunt, H.H., … Greenwald, I. (1996). Assessment of normal and mutant human presenilin function in Caenorhabditis elegans. Proceedings of the National Academy of Sciences of the United States of America, 93(25), 14940–14944. doi:10.1073/pnas.93.25.14940
   PubMed Web of Science ®Google Scholar
• Levitan, D., & Greenwald, I. (1995). Facilitation of lin-12-mediated signalling by sel-12, a Caenorhabditis elegans S182 Alzheimer’s disease gene. Nature, 377(6547), 351–354. doi:10.1038/377351a0
   PubMed Web of Science ®Google Scholar
• Liachko, N.F., Guthrie, C.R., & Kraemer, B.C. (2010). Phosphorylation promotes neurotoxicity in a Caenorhabditis elegans model of TDP-43 proteinopathy. The Journal of Neuroscience: The Official Journal of the Society for Neuroscience, 30(48), 16208–16219. doi:10.1523/JNEUROSCI.2911-10.2010
   PubMed Web of Science ®Google Scholar
• Ling, S.C., Polymenidou, M., & Cleveland, D.W. (2013). Converging mechanisms in ALS and FTD: Disrupted RNA and protein homeostasis. Neuron, 79(3), 416–438. doi:10.1016/j.neuron.2013.07.033
   PubMed Web of Science ®Google Scholar
• Link, C.D. (1995). Expression of human beta-amyloid peptide in transgenic Caenorhabditis elegans. Proceedings of the National Academy of Sciences of the United States of America, 92(20), 9368–9372. doi:10.1073/pnas.92.20.9368
   PubMed Web of Science ®Google Scholar
• Link, C.D., Taft, A., Kapulkin, V., Duke, K., Kim, S., Fei, Q., … Sahagan, B.G. (2003). Gene expression analysis in a transgenic Caenorhabditis elegans Alzheimer’s disease model. Neurobiology of Aging, 24(3), 397–413. doi:10.1016/S0197-4580(02)00224-5
   PubMed Web of Science ®Google Scholar
• Liu, Z., Hamamichi, S., Lee, B.D., Yang, D., Ray, A., Caldwell, G.A., … Dawson, V.L. (2011). Inhibitors of LRRK2 kinase attenuate neurodegeneration and Parkinson-like phenotypes in Caenorhabditis elegans and Drosophila Parkinson’s disease models. Human Molecular Genetics, 20(20), 3933–3942. doi:10.1093/hmg/ddr312
   PubMed Web of Science ®Google Scholar
• Lloret, A., Badia, M.-C., Giraldo, E., Ermak, G., Alonso, M.-D., Pallardó, F.V., … Viña, J. (2011). Amyloid-β toxicity and tau hyperphosphorylation are linked via RCAN1 in Alzheimer’s disease. Journal of Alzheimer’s Disease, 27(4), 701–709. doi:10.3233/JAD-2011-110890
   PubMed Web of Science ®Google Scholar
• Maloney, B., & Lahiri, D.K. (2011). The Alzheimer’s amyloid β-peptide (Aβ) binds a specific DNA Aβ-interacting domain (AβID) in the APP, BACE1, and APOE promoters in a sequence-specific manner: Characterizing a new regulatory motif. Gene, 488(1–2), 1–12. doi:10.1016/j.gene.2011.06.004
   PubMed Web of Science ®Google Scholar
• Martinez, B.A., Caldwell, K.A., & Caldwell, G.A. (2017). C. elegans as a model system to accelerate discovery for Parkinson disease. Current Opinion in Genetics & Development, 44, 102–109. doi:10.1016/j.gde.2017.02.011
   PubMed Web of Science ®Google Scholar
• Martinez, B.A., Petersen, D.A., Gaeta, A.L., Stanley, S.P., Caldwell, G.A., & Caldwell, K.A. (2017). Dysregulation of the mitochondrial unfolded protein response induces non-apoptotic dopaminergic neurodegeneration in C. elegans models of Parkinson’s disease. The Journal of Neuroscience: The Official Journal of the Society for Neuroscience, 37(46), 11085–11100. doi:10.1523/JNEUROSCI.1294-17.2017
   PubMed Web of Science ®Google Scholar
• Martinez, M.A.Q., Kinney, B.A., Medwig-Kinney, T.N., Ashley, G., Ragle, J.M., Johnson, L., … Matus, D.Q. (2020). Rapid degradation of Caenorhabditis elegans proteins at single-cell resolution with a synthetic auxin. G3, 10(1), 267–280. doi:10.1534/g3.119.400781
   Web of Science ®Google Scholar
• Mattson, M.P., & Magnus, T. (2006). Ageing and neuronal vulnerability. Nature Reviews. Neuroscience, 7(4), 278–294. doi:10.1038/nrn1886
   PubMed Web of Science ®Google Scholar
• McColl, G., Roberts, B.R., Gunn, A.P., Perez, K.A., Tew, D.J., Masters, C.L., … Bush, A.I. (2009). The Caenorhabditis elegans A beta 1-42 model of Alzheimer disease predominantly expresses A beta 3-42. The Journal of Biological Chemistry, 284(34), 22697–22702. doi:10.1074/jbc.C109.028514
   PubMed Web of Science ®Google Scholar
• McDermott, J.B., Aamodt, S., & Aamodt, E. (1996). ptl-1, a Caenorhabditis elegans gene whose products are homologous to the tau microtubule-associated proteins. Biochemistry, 35(29), 9415–9423.
   PubMed Web of Science ®Google Scholar
• McDiarmid, T.A., Au, V., Loewen, A., Liang, J.J.H., Mizumoto, K., Moerman, D.G., & Rankin, C.H. (2018). CRISPR-Cas9 human gene replacement and phenomic characterization in Caenorhabditis elegans to understand the functional conservation of human genes and decipher variants of uncertain significance. Disease Models and Mechanisms, 11, 1–15. 10.1101/369249
   Web of Science ®Google Scholar
• Mejzini, R., Flynn, L.L., Pitout, I.L., Fletcher, S., Wilton, S.D., & Akkari, P.A. (2019). ALS genetics, mechanisms, and therapeutics: Where are we now? Frontiers in Neuroscience, 13, 1310–1327. doi:10.3389/fnins.2019.01310
   PubMed Web of Science ®Google Scholar
• Morley, J.F., Brignull, H.R., Weyers, J.J., & Morimoto, R.I. (2002). The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans. Proceedings of the National Academy of Sciences of the United States of America, 99(16), 10417–10422. doi:10.1073/pnas.152161099
   PubMed Web of Science ®Google Scholar
• Murakami, T., Yang, S.-P., Xie, L., Kawano, T., Fu, D., Mukai, A., … St George-Hyslop, P. (2012). ALS mutations in FUS cause neuronal dysfunction and death in Caenorhabditis elegans by a dominant gain-of-function mechanism. Human Molecular Genetics, 21(1), 1–9. doi:10.1093/hmg/ddr417
   PubMed Web of Science ®Google Scholar
• Nalls, M.A., Pankratz, N., Lill, C.M., Do, C.B., Hernandez, D.G., Saad, M., … Singleton, A.B. (2014). Large-scale meta-analysis of genome-wide association data identifies six new risk loci for Parkinson’s disease. Nature Genetics, 46(9), 989–993. doi:10.1038/ng.3043
   PubMed Web of Science ®Google Scholar
• Neumann, M., Sampathu, D.M., Kwong, L.K., Truax, A.C., Micsenyi, M.C., Chou, T.T., … Lee, V.M.-Y. (2006). Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science, 314(5796), 130–133. doi:10.1126/science.1134108
   PubMed Web of Science ®Google Scholar
• Nollen, E.A.A., Garcia, S.M., van Haaften, G., Kim, S., Chavez, A., Morimoto, R.I., & Plasterk, R.H.A. (2004). Genome-wide RNA interference screen identifies previously undescribed regulators of polyglutamine aggregation. Proceedings of the National Academy of Sciences of the United States of America, 101(17), 6403–6408. doi:10.1073/pnas.0307697101
   PubMed Web of Science ®Google Scholar
• Oeda, T., Shimohama, S., Kitagawa, N., Kohno, R., Imura, T., Shibasaki, H., & Ishii, N. (2001). Oxidative stress causes abnormal accumulation of familial amyotrophic lateral sclerosis-related mutant SOD1 in transgenic Caenorhabditis elegans. Human Molecular Genetics, 10(19), 2013–2023. doi:10.1093/hmg/10.19.2013
   PubMed Web of Science ®Google Scholar
• Oliveira, J.M., Henriques, A.G., Martins, F., Rebelo, S., & da Cruz e Silva, O.A.B. (2015). Amyloid-β modulates both AβPP and tau phosphorylation. Journal of Alzheimer’s Disease, 45(2), 495–507. doi:10.3233/JAD-142664
   PubMed Web of Science ®Google Scholar
• Paisán-Ruíz, C., Jain, S., Evans, E.W., Gilks, W.P., Simón, J., van der Brug, M., … Singleton, A.B. (2004). Cloning of the gene containing mutations that cause PARK8-linked Parkinson’s disease. Neuron, 44(4), 595–600. doi:10.1016/j.neuron.2004.10.023
   PubMed Web of Science ®Google Scholar
• Parker, J.A., Arango, M., Abderrahmane, S., Lambert, E., Tourette, C., Catoire, H., & Néri, C. (2005). Resveratrol rescues mutant polyglutamine cytotoxicity in nematode and mammalian neurons. Nature Genetics, 37(4), 349–350. doi:10.1038/ng1534
   PubMed Web of Science ®Google Scholar
• Parker, J.A., Connolly, J.B., Wellington, C., Hayden, M., Dausset, J., & Neri, C. (2001). Expanded polyglutamines in Caenorhabditis elegans cause axonal abnormalities and severe dysfunction of PLM mechanosensory neurons without cell death. Proceedings of the National Academy of Sciences of the United States of America, 98(23), 13318–13323. doi:10.1073/pnas.231476398
   PubMed Web of Science ®Google Scholar
• Parker, J.A., Metzler, M., Georgiou, J., Mage, M., Roder, J.C., Rose, A.M., … Néri, C. (2007). Huntingtin-interacting protein 1 influences worm and mouse presynaptic function and protects Caenorhabditis elegans neurons against mutant polyglutamine toxicity. The Journal of Neuroscience: The Official Journal of the Society for Neuroscience, 27(41), 11056–11064. doi:10.1523/JNEUROSCI.1941-07.2007
   PubMed Web of Science ®Google Scholar
• Puls, I., Jonnakuty, C., LaMonte, B.H., Holzbaur, E.L.F., Tokito, M., Mann, E., … Fischbeck, K.H. (2003). Mutant dynactin in motor neuron disease. Nature Genetics, 33(4), 455–456. doi:10.1038/ng1123
   PubMed Web of Science ®Google Scholar
• Ramirez, A., Heimbach, A., Gründemann, J., Stiller, B., Hampshire, D., Cid, L.P., … Kubisch, C. (2006). Hereditary Parkinsonism with dementia is caused by mutations in ATP13A2, encoding a lysosomal type 5 P-type ATPase. Nature Genetics, 38(10), 1184–1191. doi:10.1038/ng1884
   PubMed Web of Science ®Google Scholar
• Ramonet, D., Podhajska, A., Stafa, K., Sonnay, S., Trancikova, A., Tsika, E., … Moore, D.J. (2012). PARK9-associated ATP13A2 localizes to intracellular acidic vesicles and regulates cation homeostasis and neuronal integrity. Human Molecular Genetics, 21(8), 1725–1743. doi:10.1093/hmg/ddr606
   PubMed Web of Science ®Google Scholar
• Reiner, A., Dragatsis, I., & Dietrich, P. (2011). Genetics and neuropathology of Huntington’s disease. International Review of Neurobiology, 98, 325–372. doi:10.1016/B978-0-12-381328-2.00014-6
   Google Scholar
• Renton, A.E., Majounie, E., Waite, A., Simón-Sánchez, J., Rollinson, S., Gibbs, J.R., … Traynor, B.J. (2011). A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron, 72(2), 257–268. doi:10.1016/j.neuron.2011.09.010
   PubMed Web of Science ®Google Scholar
• Rha, J., Jones, S.K., Fidler, J., Banerjee, A., Leung, S.W., Morris, K.J., … Corbett, A.H. (2017). The RNA-binding protein, ZC3H14, is required for proper poly(A) tail length control, expression of synaptic proteins, and brain function in mice. Human Molecular Genetics, 26(19), 3663–3681. doi:10.1093/hmg/ddx248
   PubMed Web of Science ®Google Scholar
• Rosen, D.R., Siddique, T., Patterson, D., Figlewicz, D.A., Sapp, P., Hentati, A., … Deng, H.X. (1993). Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature, 362(6415), 59–62. doi:10.1038/362059a0
   PubMed Web of Science ®Google Scholar
• Rudich, P., Snoznik, C., Watkins, S.C., Monaghan, J., Pandey, U.B., & Lamitina, S.T. (2017). Nuclear localized C9orf72-associated arginine-containing dipeptides exhibit age-dependent toxicity in C. elegans. Human Molecular Genetics, 26(24), 4916–4928. doi:10.1093/hmg/ddx372
   PubMed Web of Science ®Google Scholar
• Saha, S., Guillily, M.D., Ferree, A., Lanceta, J., Chan, D., Ghosh, J., … Wolozin, B. (2009). LRRK2 modulates vulnerability to mitochondrial dysfunction in Caenorhabditis elegans. The Journal of Neuroscience: The Official Journal of the Society for Neuroscience, 29(29), 9210–9218. doi:10.1523/JNEUROSCI.2281-09.2009
   PubMed Web of Science ®Google Scholar
• Şahin, A., Held, A., Bredvik, K., Major, P., Achilli, T.-M., Kerson, A.G., … Reenan, R. (2017). Human SOD1 ALS mutations in a Drosophila knock-in model cause severe phenotypes and reveal dosage-sensitive gain- and loss-of-function components. Genetics, 205(2), 707–723. doi:10.1534/genetics.116.190850
   PubMed Web of Science ®Google Scholar
• Sakaguchi-Nakashima, A., Meir, J.Y., Jin, Y., Matsumoto, K., & Hisamoto, N. (2007). LRK-1, a C. elegans PARK8-related kinase, regulates axonal-dendritic polarity of SV proteins. Current Biology, 17(7), 592–598. doi:10.1016/j.cub.2007.01.074
   PubMed Web of Science ®Google Scholar
• Sämann J., Hegermann J., von Gromoff E., Eimer S., Baumeister R. & Schmidt E. (2009) Caenorhabditis elegans LRK-1 and PINK-1 Act Antagonistically in Stress Response and Neurite Outgrowth. Journal of Biological Chemistry, 284(24), 16482–16491.
   Google Scholar
• Sarasija, S., Laboy, J.T., Ashkavand, Z., Bonner, J., Tang, Y., & Norman, K.R. (2018). Presenilin mutations deregulate mitochondrial Ca2+ homeostasis and metabolic activity causing neurodegeneration in Caenorhabditis elegans. eLife, 7, 1–30. doi:10.7554/eLife.33052
   Web of Science ®Google Scholar
• Sarasija, S., & Norman, K.R. (2015). A γ-Secretase independent role for presenilin in calcium homeostasis impacts mitochondrial function and morphology in Caenorhabditis elegans. Genetics, 201(4), 1453–1466. doi:10.1534/genetics.115.182808
   PubMed Web of Science ®Google Scholar
• Satyal, S.H., Schmidt, E., Kitagawa, K., Sondheimer, N., Lindquist, S., Kramer, J.M., & Morimoto, R.I. (2000). Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans. Proceedings of the National Academy of Sciences of the United States of America, 97(11), 5750–5755. doi:10.1073/pnas.100107297
   PubMed Web of Science ®Google Scholar
• Saudou, F., & Humbert, S. (2016). The biology of huntingtin. Neuron, 89(5), 910–926. doi:10.1016/j.neuron.2016.02.003
   PubMed Web of Science ®Google Scholar
• Sawa, A., Tomoda, T., & Bae, B.-I. (2003). Mechanisms of neuronal cell death in Huntington’s disease. Cytogenetic and Genome Research, 100(1–4), 287–295. doi:10.1159/000072864
   PubMed Web of Science ®Google Scholar
• Selkoe, D.J., & Hardy, J. (2016). The amyloid hypothesis of Alzheimer’s disease at 25 years. EMBO Molecular Medicine, 8(6), 595–608. doi:10.15252/emmm.201606210
   PubMed Web of Science ®Google Scholar
• Springer, W., Hoppe, T., Schmidt, E., & Baumeister, R. (2005). A Caenorhabditis elegans Parkin mutant with altered solubility couples alpha-synuclein aggregation to proteotoxic stress. Human Molecular Genetics, 14(22), 3407–3423. doi:10.1093/hmg/ddi371
   PubMed Web of Science ®Google Scholar
• Sulston, J.E., & Horvitz, H.R. (1977). Post-embryonic cell lineages of the nematode, Caenorhabditis elegans. Developmental Biology, 56(1), 110–156. doi:10.1016/0012-1606(77)90158-0
   PubMed Web of Science ®Google Scholar
• Sulston, J.E., Schierenberg, E., White, J.G., & Thomson, J.N. (1983). The embryonic cell lineage of the nematode Caenorhabditis elegans. Developmental Biology, 100(1), 64–119. doi:10.1016/0012-1606(83)90201-4
   PubMed Web of Science ®Google Scholar
• Tabaton, M., Zhu, X., Perry, G., Smith, M.A., & Giliberto, L. (2010). Signaling effect of amyloid-beta(42) on the processing of AbetaPP. Experimental Neurology, 221(1), 18–25. doi:10.1016/j.expneurol.2009.09.002
   PubMed Web of Science ®Google Scholar
• Tafuri, F., Ronchi, D., Magri, F., Comi, G.P., & Corti, S. (2015). SOD1 misplacing and mitochondrial dysfunction in amyotrophic lateral sclerosis pathogenesis. Frontiers in Cellular Neuroscience, 9, 336–312. doi:10.3389/fncel.2015.00336
   PubMed Web of Science ®Google Scholar
• Takashima, A., Noguchi, K., Michel, G., Mercken, M., Hoshi, M., Ishiguro, K., & Imahori, K. (1996). Exposure of rat hippocampal neurons to amyloid β peptide (25–35) induces the inactivation of phosphatidyl inositol-3 kinase and the activation of tau protein kinase I/glycogen synthase kinase-3β. Neuroscience Letters, 203(1), 33–36. doi:10.1016/0304-3940(95)12257-5
   PubMed Web of Science ®Google Scholar
• Teo, E., Ravi, S., Barardo, D., Kim, H.-S., Fong, S., Cazenave-Gassiot, A., … Gruber, J. (2019). Metabolic stress is a primary pathogenic event in transgenic Caenorhabditis elegans expressing pan-neuronal human amyloid beta. eLife, 8, 1–25. doi:10.7554/eLife.50069
   Web of Science ®Google Scholar
• The C. elegans Sequencing Consortium. (1998). Genome sequence of the nematode C. elegans: A platform for investigating biology. Science, 282(5396), 2012–2018. 10.1126/science.282.5396.2012
   PubMed Web of Science ®Google Scholar
• Therrien, M., & Parker, J.A. (2014). Worming forward: Amyotrophic lateral sclerosis toxicity mechanisms and genetic interactions in Caenorhabditis elegans. Frontiers in Genetics, 5, 85. doi:10.3389/fgene.2014.00085
   PubMed Web of Science ®Google Scholar
• Therrien, M., Rouleau, G.A., Dion, P.A., & Parker, J.A. (2013). Deletion of C9ORF72 results in motor neuron degeneration and stress sensitivity in C. elegans. PLoS One, 8(12), e83450. doi:10.1371/journal.pone.0083450
   PubMed Web of Science ®Google Scholar
• Timmons, L., Tabara, H., Mello, C.C., & Fire, A.Z. (2003). Inducible systemic RNA silencing in Caenorhabditis elegans. Molecular Biology of the Cell, 14(7), 2972–2983. doi:10.1091/mbc.e03-01-0858
   PubMed Web of Science ®Google Scholar
• Turner, M.R., Hardiman, O., Benatar, M., Brooks, B.R., Chio, A., de Carvalho, M., … Kiernan, M.C. (2013). Controversies and priorities in amyotrophic lateral sclerosis. The Lancet Neurology, 12(3), 310–322. doi:10.1016/S1474-4422(13)70036-X
   PubMed Web of Science ®Google Scholar
• Vaccaro, A., Patten, S.A., Aggad, D., Julien, C., Maios, C., Kabashi, E., … Parker, J.A. (2013). Pharmacological reduction of ER stress protects against TDP-43 neuronal toxicity in vivo. Neurobiology of Disease, 55, 64–75. doi:10.1016/j.nbd.2013.03.015
   PubMed Web of Science ®Google Scholar
• Vaccaro, A., Tauffenberger, A., Aggad, D., Rouleau, G., Drapeau, P., & Parker, J.A. (2012). Mutant TDP-43 and FUS cause age-dependent paralysis and neurodegeneration in C. elegans. PLoS One, 7(2), e31321. doi:10.1371/journal.pone.0031321
   PubMed Web of Science ®Google Scholar
• Vaccaro, A., Tauffenberger, A., Ash, P.E.A., Carlomagno, Y., Petrucelli, L., & Parker, J.A. (2012). TDP-1/TDP-43 regulates stress signaling and age-dependent proteotoxicity in Caenorhabditis elegans. PLoS Genetics, 8(7), e1002806. doi:10.1371/journal.pgen.1002806
   PubMed Web of Science ®Google Scholar
• Vérièpe, J., Fossouo, L., & Parker, J.A. (2015). Neurodegeneration in C. elegans models of ALS requires TIR-1/Sarm1 immune pathway activation in neurons. Nature Communications, 6. doi:10.1038/ncomms8319
   PubMed Web of Science ®Google Scholar
• Wallings, R., Manzoni, C., & Bandopadhyay, R. (2015). Cellular processes associated with LRRK2 function and dysfunction. The FEBS Journal, 282(15), 2806–2826. doi:10.1111/febs.13305
   PubMed Web of Science ®Google Scholar
• Wang, C., Saar, V., Leung, K.L., Chen, L., & Wong, G. (2018). Human amyloid β peptide and tau co-expression impairs behavior and causes specific gene expression changes in Caenorhabditis elegans. Neurobiology of Disease, 109, 88–101. doi:10.1016/j.nbd.2017.10.003
   PubMed Web of Science ®Google Scholar
• Wang, J., Farr, G.W., Hall, D.H., Li, F., Furtak, K., Dreier, L., & Horwich, A.L. (2009). An ALS-linked mutant SOD1 produces a locomotor defect associated with aggregation and synaptic dysfunction when expressed in neurons of Caenorhabditis elegans. PLoS Genetics, 5(1), e1000350. doi:10.1371/journal.pgen.1000350
   PubMed Web of Science ®Google Scholar
• Wang, S., Tang, N.H., Lara-Gonzalez, P., Zhao, Z., Cheerambathur, D.K., Prevo, B., … Oegema, K. (2017). A toolkit for GFP-mediated tissue-specific protein degradation in C. elegans. Development, 144(14), 2694–2701. doi:10.1242/dev.150094
   PubMed Web of Science ®Google Scholar
• White, J.G., Southgate, E., Thomson, J.N., & Brenner, S. (1986). The structure of the nervous system of the nematode Caenorhabditis elegans. Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences, 314(1165), 1–340. doi:10.1098/rstb.1986.0056
   PubMed Web of Science ®Google Scholar
• Wiese M., Antebi A., Zheng H. (2010). Intracellular Trafficking and Synaptic Function of APL-1 in Caenorhabditis elegans. PLoS One, 5(9), e12790.
   Google Scholar
• Williamson, T.L., & Cleveland, D.W. (1999). Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neurons. Nature Neuroscience, 2(1), 50–56. doi:10.1038/4553
   PubMed Web of Science ®Google Scholar
• Wils, H., Kleinberger, G., Janssens, J., Pereson, S., Joris, G., Cuijt, I., … Kumar-Singh, S. (2010). TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration. Proceedings of the National Academy of Sciences of the United States of America, 107(8), 3858–3863. doi:10.1073/pnas.0912417107
   PubMed Web of Science ®Google Scholar
• Xia, Q., Wang, H., Hao, Z., Fu, C., Hu, Q., Gao, F., … Wang, G. (2016). TDP-43 loss of function increases TFEB activity and blocks autophagosome-lysosome fusion. The EMBO Journal, 35(2), 121–142. doi:10.15252/embj.201591998
   PubMed Web of Science ®Google Scholar
• Xu, Y.-F., Gendron, T.F., Zhang, Y.-J., Lin, W.-L., D’Alton, S., Sheng, H., … Petrucelli, L. (2010). Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice. The Journal of Neuroscience: The Official Journal of the Society for Neuroscience, 30(32), 10851–10859. doi:10.1523/JNEUROSCI.1630-10.2010
   PubMed Web of Science ®Google Scholar
• Yanase, S., Onodera, A., Tedesco, P., Johnson, T.E., & Ishii, N. (2009). SOD-1 deletions in Caenorhabditis elegans alter the localization of intracellular reactive oxygen species and show molecular compensation. The Journals of Gerontology. Series A, Biological Sciences and Medical Sciences, 64(5), 530–539. doi:10.1093/gerona/glp020
   PubMed Web of Science ®Google Scholar
• Yao, C., Johnson, W.M., Gao, Y., Wang, W., Zhang, J., Deak, M., … Chen, S.G. (2013). Kinase inhibitors arrest neurodegeneration in cell and C. elegans models of LRRK2 toxicity. Human Molecular Genetics, 22(2), 328–344. doi:10.1093/hmg/dds431
   PubMed Web of Science ®Google Scholar
• Yim, M.B., Kang, J.H., Yim, H.S., Kwak, H.S., Chock, P.B., & Stadtman, E.R. (1996). A gain-of-function of an amyotrophic lateral sclerosis-associated Cu,Zn-superoxide dismutase mutant: An enhancement of free radical formation due to a decrease in Km for hydrogen peroxide. Proceedings of the National Academy of Sciences of the United States of America, 93(12), 5709–5714. doi:10.1073/pnas.93.12.5709
   PubMed Web of Science ®Google Scholar
• Zhang, L., Ward, J.D., Cheng, Z., & Dernburg, A.F. (2015). The auxin-inducible degradation (AID) system enables versatile conditional protein depletion in C. elegans. Development, 142(24), 4374–4384. doi:10.1242/dev.129635
   PubMed Web of Science ®Google Scholar
• Zhang, S., Cai, F., Wu, Y., Bozorgmehr, T., Wang, Z., Zhang, S., … Song, W. (2020). A presenilin-1 mutation causes Alzheimer disease without affecting Notch signaling. Molecular Psychiatry, 25 (3), 603–611. doi:10.1038/s41380-018-0101-x
   PubMed Web of Science ®Google Scholar
• Zhang, T., Hwang, H.-Y., Hao, H., Talbot, C., & Wang, J. (2012). Caenorhabditis elegans RNA-processing protein TDP-1 regulates protein homeostasis and life span. The Journal of Biological Chemistry, 287(11), 8371–8382. doi:10.1074/jbc.M111.311977
   PubMed Web of Science ®Google Scholar
• Zhang, T., Mullane, P.C., Periz, G., & Wang, J. (2011). TDP-43 neurotoxicity and protein aggregation modulated by heat shock factor and insulin/IGF-1 signaling. Human Molecular Genetics, 20(10), 1952–1965. doi:10.1093/hmg/ddr076
   PubMed Web of Science ®Google Scholar
• Zheng, J., Winderickx, J., Franssens, V., & Liu, B. (2018). A mitochondria-associated oxidative stress perspective on Huntington’s disease. Frontiers in Molecular Neuroscience, 11, 329–310. doi:10.3389/fnmol.2018.00329
   PubMed Web of Science ®Google Scholar
• Zou, K., Gong, J.S., Yanagisawa, K., & Michikawa, M. (2002). A novel function of monomeric amyloid β-protein serving as an antioxidant molecule against metal-induced oxidative damage. The Journal of Neuroscience: The Official Journal of the Society for Neuroscience, 22(12), 4833–4841. doi:10.1523/JNEUROSCI.22-12-04833.2002
   PubMed Web of Science ®Google Scholar