2021

Chang, K., Agrawal, I., Vainshtein, A., Ho, W., Xin, W., Tucker-Kellogg, G., Susuki, K., Peles, E., Ling, S. and Chan, J., "TDP-43 maximizes nerve conduction velocity by repressing a cryptic exon for paranodal junction assembly in Schwann cells." eLife 2021, 10:e64456.

Ho WY, Agrawal I, Tyan SH, Sanford E, Chang WT, Lim K, Ong J, Tan BSY, Moe AAK, Yu R, Wong P, Tucker-Kellogg G, Koo E, Chuang KH, Ling SC. "Dysfunction in nonsense-mediated decay, protein homeostasis, mitochondrial function, and brain connectivity in ALS-FUS mice with cognitive deficits." Acta Neuropathol Commun 2021, 9: 9 .

2020

Peng, A. Y., Agrawal, I., Ho, W. Y., Yen, Y., Pinter, A. J., Liu, J., Phua, Q. C., Koh, K., Chang, JC., Sandford, E., Man, J., Wong, P., Gutmann, D. H., Tucker-Kellogg, G., Ling, S. “Loss of TDP-43 in astrocytes leads to motor deficits by triggering A1-like reactive phenotype and triglial dysfunction”, Proceedings of the National Academy of Sciences, Oct 2020, 202007806

Ho WY., Gangadharan, SN., Liu F., Soong TW., Ling, S.-C., “Deregulated expression of a longevity gene, Klotho, in the C9orf72 deletion mice with impaired synaptic plasticity and adult neurogenesis in the hippocampus”, Acta Neuropathol Commun 2020 8:1-7

Wong, P., Ho, W.Y., Yen Y.-C., Sanford, E., Ling, S.-C.*, "The vulnerability of motor and frontal cortex- dependent behaviors in mice expressing ALS-linked mutation in TDP-43”, Neurobiology of Aging, 2020, 92: 43-60, *corresponding author

2019

Ho, W.Y., Tyan, S.-H., Chang, W.T., Yen, Y.C. Lim, K, Tan, B.S.Y., Ong, J., Wong, P., Tucker-Kellogg, G., Koo, E., Ling, S.-C*., “FUS-mediated down-regulation of Sema5a, an autism-related gene, in FUS mice with hippocampus-dependent cognitive deficits", Hum Mol Gene, 2019, 22: 3777–3791, *corresponding author

Cali, C. P., Patino, M., Tai, Y. T., Ho, W. Y., PSP Genetics Consortium, Ghetti, B., Van Deerlin, V. M., Lee, V. M., Trojanowski, J. Q., Mok, K. Y., Ling, H., Dickson, D. W., Schellenberg, G. D., Ling, S.-C. and Lee, E. B., “C9orf72 Intermediate Repeats Confer Risk for Corticobasal Degeneration via increased C9orf72 Expression and disruption of autophagy", Acta Neuropathologica, 2019, 14: 26-42

Ho, W.Y., Tai, Y.K., Cherg, J-C., Liang, L., Tyan, S.-H., Chen, S., Guan, J.-L., Zhuo, H., Shen, H.-M., Koo, E., Ling, S.-C.*, “ALS/FTD-linked mutation in FUS suppresses intra-axonal protein synthesis and drives motor neuron and FTD like-disease without nuclear loss-of-functions of FUS", Autophagy, 2019, 15(5): 827-842. *corresponding author

Ling, S.-C.*, Dastidar, S. G., Tokunaga, S., Ho, W. Y., Lim, K., Ilieva, H., Parone, P. A., Tyan, S. H., Platoshyn, O., Bui, N. B., Bui, A. T., Vetto, A. P., Sun, S., McAlonis-Downes, M., Han, J. S., Swing, D., Kapeli, K., Yeo, G. W., Tessarollo, L., Marsala, M., Tucker-Kellogg, G., Shaw, C. E., La Spada, A. R., Lagier-Tourenne, C., Da Cruz, S., and Cleveland, D. W., “Overriding FUS autoregulation triggers gain-of-toxic dysfunctions in autophagy-lysosome axis and RNA metabolism", eLife, 2019, 8:602, *co-corresponding author

2018

Ling, S.-C.*, “Synaptic paths to neurodegeneration: the emerging role of TDP-43 and FUS in synaptic functions", Neural Plasticity, 2018, doi: 10.1155/2018/8413496, *: corresponding author

Bennet, C., Dastidar, S., Ling, S.-C., Malik, B., Ashe, T., Miller, D., Van Es, M., Matthew., M., Lee., C., Chen., Y. Sopher, B., Cleveland, D., La Spada, A., “Senataxin mutations elicit motor neuron degeneration phenotype and yield TDP-43 misloclaization in ALS4 mice and human patients", Acta Neuropathologica, 2018, 136(3):425-443

Lu, W., Lakonishok, M., Serpinskaya, A. S., Ling, S.-C., Gelfand, V. I., “Ooplasmic flow cooperates with transport and anchorage in Drosophila oocyte posterior determination", J. Cell Biol., 2018, 217(10):3497-3511

Lopez-Erauskin, J., Myers, B., McAlonis-Downes, M., Chillon-Marinas, C., Bui, A.T., Vetto, A. P., Lee, S. K., Le, A. Y., Sun, Y., Baughn, M., Jambeau, M., Artates, J., Boubaker, J., Hicks, G. G., Swing, D., Qiu, J., Ouyang, Z., Fu, X. D., Tessarollo, L., Ling, S.-C., Parone, P. A., Shaw, C. E., Lagier-Tourenne, C., Cleveland, D. W., and Da Cruz, S., “ALS/FTD-linked mutation in FUS suppresses intra-axonal protein synthesis and drives motor neuron and FTD like-disease without nuclear loss-of-functions of FUS", Neuron, 2018, 100(4):816-830

Wang, J., Ho, H.W., Lim, K., Feng, J., Tucker-Kellogg, G., Nave., K.-A., Ling, S.-C.*, “Cell-autonomous requirement of TDP-43, an ALS/FTD signature protein, for oligodendrocyte survival and myelination", Proc Natl Acad Sci U S A., 2018, 115(46):E10941-10950, *: corresponding author

2017

Ditsworth, D., Maldonado, M., McAlonis-Downes, M., Sun, S., Seelman, A., Drenner, K., Arnold, E., Ling, S.-C., Pizzo, D., Ravits, J., Cleveland, D. W., Da Cruz, S., “Mutant TDP-43 within motor neurons drive disease onset but not progressive in Amyotrophic Lateral Sclerosis" Acta Neuropathologica, 2017(351):602

2016

Jiang, J., Zhu, Q., Gendron, T. F., Saberi, F., Staufer, J., Jafar-nejad, P., Drenner, K., Schulte, D., Chun, S., Seelman, A., Sun, S., Ling, S.-C., McAlonis-Downes, M., Myers, B., Baughn, M., Platoshyn, O., Marsala, M., Watt, A., Heyser, C., Ard, C., De Muynck, L., Daughrity, L., Swing, D., Tessarollo, L., de Jong, P., Edbauer, D., Van Damme, P., Petrucelli, L., Shaw, C. E., Bennett, C. F., Da Cruz, S., Ravits, J., Rigo, F., Cleveland, D. W., and Lagier-Tourenne, C., "Antisense oligonucleotide therapy for amyotrophic lateral sclerosis and frontal temporal dementia caused by a gain of toxicity from hexanucleotide expansions in the C9orf72 gene", Neuron, 2016 (90): 1-16, Impact factor: 15.054

Seredenina, T., Nayernia, Z., Maghzal, G. J., Filippova, A., Sorce, I., Ling, S.-C., Basset, O., Plastre, O., Daali, Y., Rushing, E. J., Cleveland, D. W., Aguzzi, A., Stocker, R., Krause K.-H., Jaquet, V., "The NOX-inhibitory phenothiazine thioridazine decreases neuroinflammation and transiently improves motor function in a familial model of amyotrophic lateral sclerosis", Free Radical Biology and Medicine, 2016 (87): 95-108. Impact factor: 5.736

Tio, M., Wen, R., Lim, Y. L., Wang, H., Ling, S.-C., Zhao, Y., Tan, E. K., “FUS-linked essential tremor associated with motor dysfuction in Drosophila”, Hum Genet, 2016 (135): 1223-1232. Impact factor: 4.633

2015

Mitchell, J. C., Constable, R., So, E., Vance, C., Scotter, E., Glover, L., Hortobagyi, T., Arnold, E. S., Ling, S.-C., McAlonis, M., Da Cruz, S., Polymenidou, M., Tessarolo, L., Cleveland, D. W., Shaw, C. E., “Wild type human TDP-43 potentiateds ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS.” Acta Neuropathol Commun

Sun, S.*, Ling, S.-C*., Qiu, J., Albuquerque, C., Zhou, Y., Tokunaga, S., Li, H., Qiu, H., Bui, A., Yeo, G. W., Huang, E. J., Eggan, K., Zhou, H., Fu., X.-D., Lagier-Tourenne, C., and Cleveland, D. W., “ALS-causative mutations in FUS/TLS confer both gain- and loss-of RNA-processing functions by altered association with SMN and U1-snRNP.” Nature Communication, 2015 (6):6171 * co-first author

Sun S., Sun Y., Ling, S.-C., Ferraiuolo L., McAlonis-Downes M., Zou Y., Drenner K., Wang Y., Ditsworth D., Tokunaga S., Kopelevich A., Kaspar, B.K., Lagier-Tourenne, C., and Cleveland, D. W., “Translational profiling identifies a cascade of damage initiated in motor neurons and spreading to glia in mutant SOD1-mediated ALS.” Proc Natl Acad Sci U S A., 2015 112(50): E6993-7002. Impact factor: 9.674

2014

Cortes, C. J., Ling, S.-C., Gou, L.T., Hung, G., Tsunemi, T., Ly, L., Tokunaga, S., Lopez, E., Sopher, B. L., Bennett, C. F., Shelton, G. D., Cleveland, D. W., and La Spada, A. R., “Muscle expression of mutant androgen receptor accounts for systemic and motor neuron disease phenotypes in spinal and bulbar muscular atrophy..” Neuron, 2014: 295-307

2013

Lagier-Tourenne, C., Baughn, M., Rigo, F., Sun, S., Liu, P., Li, H.-R., Jiang, J., Watt, A., Chun, S., Katz, M., Qiu, J., Ling, S.-C., Zhu, Q., Polymenidou, M., Drenner, K., Artates, J. W., McAlonis M. M., Hung, G., Markmiller, S., Hutt, K. R., Pizzo, D., Baloh, R. H., Vandenberg, S., Yeo, G. W., Fu, X.-D., Bennett, C. F., Cleveland, D. W., and Ravits, J., “Targeted degradation of sense and antisense C9ORF72 nuclear RNA foci as therapy for amyotrophic lateral sclerosis and frontotemporal dementia.” Proc Natl Acad Sci U S A., 2013: E4530-9

Shelkovnikova, T. A., Peters, O. M., Deykin, A. V., Connor-Robson, N., Robinson, H., Ustyugov, A. A., Bachurin, S.O., Ermolkevich, T.G., Goldman, I. L., Sadchikova, E. R., Kovrazhkina, E. A., Skvortsova, V. I., Ling, S.-C., Da Cruz, S., Parone, A. A., Buchman, V. L., and Ninkina, N. N., “Fused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA-binding motifs triggers proteinopathy and severe motor phenotype in transgenic mice.” J Biol Chem 2013: 25266-25274

Ling, S.-C., Polymenidou M., and Cleveland, D. W., “Converging mechanisms in ALS and FTLD: disrupted RNA or protein homeostasis.” Neuron, 2013: 416-438

Arnold, E.*, Ling, S.-C*., Lagier-Tourenne C., Polymenidou M., Huelga S., Ditsworth, D., Kordasiewicz, H. B., McAlonis-Downes, M., Platoshyn, O., Parone, P., Da Cruz, S., Swing, D. Tessarollo, L., Marsala, M., Shaw, C. E., Yeo, Y., Cleveland, D. W., “ALS-linked TDP-43 mutantions produce aberrant RNA splicing and adult-onset motor disease without aggregation or loss of nuclear TDP-43.” Proc Natl Acad Sci U S A, 2013: E736-745. * Co-first author

2012

Lagier-Tourenne, C., Polymenidou, M., Hutt, K. R., Vu, A. Q., Clutario, K., Baughn, M., Huelga, S. C., Ling, S.-C., Liang, T. Y., Mazur, C. Wancewicz, E., Watt, A., Freier, S., Hicks, G. G., Donohue, J. P., Shiue, L., Bennett, C. F., Ravits, J., Cleveland, D. W., and Yeo, G. W., “Convergent roles of FUS/TLS and TDP-43 in processing RNAs with long introns.” Nature Neurosci. 2012: 1488-1497

2011

Polymenidou M., Lagier-Tourenne C., Hutt K. R., Huelga S. C., Moran J., Liang T. Y., Ling S.-C., Sun E., Wancewicz E., Mazur C., Kordasiewicz, H., Sedaghat Y., Donohue J. P., Shiu L., Bennett C. F., Yeo G. W., Cleveland D. W., “Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43.” Nature Neurosci. 2011: 459-468

2010 & Before

Ling S.-C., Albuquerque, C., Han, J.-S., Lagier-Tourenne, C., Tokunaga, S., Zhou, H., Cleveland, D. W., “ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS.” Proc Natl Acad Sci U S A., 2010: 13318-13323

Kim H., Ling S.-C., Rogers G. C., Kural C., Selvin P., Rogers S. L., and Gelfand V. I., “Microtubule binding by dynactin is required for microtubule organization but not cargo transport.” J. Cell Biol . 2007: 641-651

Angenstein F., Evans A. M., Ling S.-C., Settlage R. E., Ficarro S., Carrero-Martinez F. A., Shabanowitz J., Hunt D. F., Greenough W. T. “Proteomic characterization of mRNP-complexes bound to nontranslated or translated polyA-mRNAs in the rat cerebral cortex” J. Biol. Chem. 2005: 6496-6503

Ling S.-C., Fahrner P. S., Greenough W. T., Gelfand V. I. “Transport of Drosophila fragile X mental retardation protein-containing ribonucleoprotein granules by kinesin-1 and cytoplasmic dynein” Proc Natl Acad Sci U S A. 2004 :17428-17433

Angenstein F., Evans A. M., Settlage R. E., Moran S. T., Ling S.-C., Klintsova A. Y., Shabanowitz J., Hunt D. F., Greenough W. T., “A receptor for activated C kinase is part of messenger ribonucleoprotein complexes associated with polyA-mRNAs in neurons” J. Neurosci. 2002: 8827-37

Lai S. L., Ling, S.-C., Kuo, L. H., Shu, Y. C., Chow, W. Y. and Chang, Y. C., “Characterization of granular particles isolated from postsynaptic densities” J. Neurochem. 1998: 1694-1701