Jeffrey D Rothstein

Professor

1979 …2020

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Article
2020

Antibody Therapy Targeting RAN Proteins Rescues C9 ALS/FTD Phenotypes in C9orf72 Mouse Model

Nguyen, L., Montrasio, F., Pattamatta, A., Tusi, S. K., Bardhi, O., Meyer, K. D., Hayes, L., Nakamura, K., Banez-Coronel, M., Coyne, A., Guo, S., Laboissonniere, L. A., Gu, Y., Narayanan, S., Smith, B., Nitsch, R. M., Kankel, M. W., Rushe, M., Rothstein, J., Zu, T. & 2 others, Grimm, J. & Ranum, L. P. W., Feb 19 2020, In : Neuron. 105, 4, p. 645-662.e11

Research output: Contribution to journalArticle

Astrocyte Diversity: Current Insights and Future Directions

Westergard, T. & Rothstein, J. D., Jan 1 2020, (Accepted/In press) In : Neurochemical Research.

Research output: Contribution to journalArticle

C9orf72 arginine-rich dipeptide repeat proteins disrupt karyopherin-mediated nuclear import

Hayes, L. R., Duan, L., Bowen, K., Kalab, P. & Rothstein, J. D., Mar 2020, In : eLife. 9, e51685.

Research output: Contribution to journalArticle

Open Access

Hydrogen peroxide triggers an increase in cell surface expression of system xc in cultured human glioma cells

Chase, L. A., VerHeulen Kleyn, M., Schiller, N. T., King, A. G., Flores, G., Engelsman, S. B., Bowles, C., Smith, S. L., Robinson, A. E. & Rothstein, J., Mar 2020, In : Neurochemistry International. 134, 104648.

Research output: Contribution to journalArticle

2019

Aberrant deposition of stress granule-resident proteins linked to C9orf72-associated TDP-43 proteinopathy

Chew, J., Cook, C., Gendron, T. F., Jansen-West, K., Del Rosso, G., Daughrity, L. M., Castanedes-Casey, M., Kurti, A., Stankowski, J. N., Disney, M. D., Rothstein, J. D., Dickson, D. W., Fryer, J. D., Zhang, Y. J. & Petrucelli, L., Feb 15 2019, In : Molecular neurodegeneration. 14, 1, 1 p.

Research output: Contribution to journalArticle

CRISPR-Cas9 Screens Identify the RNA Helicase DDX3X as a Repressor of C9ORF72 (GGGGCC)n Repeat-Associated Non-AUG Translation

Cheng, W., Wang, S., Zhang, Z., Morgens, D. W., Hayes, L. R., Lee, S., Portz, B., Xie, Y., Nguyen, B. V., Haney, M. S., Yan, S., Dong, D., Coyne, A. N., Yang, J., Xian, F., Cleveland, D. W., Qiu, Z., Rothstein, J. D., Shorter, J., Gao, F. B. & 2 others, Bassik, M. C. & Sun, S., Dec 4 2019, In : Neuron. 104, 5, p. 885-898.e8

Research output: Contribution to journalArticle

FGF family members differentially regulate maturation and proliferation of stem cell-derived astrocytes

Savchenko, E., Teku, G. N., Boza-Serrano, A., Russ, K., Berns, M., Deierborg, T., Lamas, N. J., Wichterle, H., Rothstein, J., Henderson, C. E., Vihinen, M. & Roybon, L., Dec 1 2019, In : Scientific reports. 9, 1, 9610.

Research output: Contribution to journalArticle

Open Access

Molecularly defined cortical astroglia subpopulation modulates neurons via secretion of Norrin

Miller, S. J., Philips, T., Kim, N., Dastgheyb, R., Chen, Z., Hsieh, Y. C., Daigle, J. G., Datta, M., Chew, J., Vidensky, S., Pham, J. T., Hughes, E. G., Robinson, M. B., Sattler, R., Tomer, R., Suk, J. S., Bergles, D. E., Haughey, N., Pletnikov, M., Hanes, J. S. & 1 others, Rothstein, J. D., Jan 1 2019, In : Nature neuroscience.

Research output: Contribution to journalArticle

Monocarboxylate transporter 1 in Schwann cells contributes to maintenance of sensory nerve myelination during aging

Jha, M. K., Lee, Y., Russell, K. A., Yang, F., Dastgheyb, R. M., Deme, P., Ament, X. H., Chen, W., Liu, Y., Guan, Y., Polydefkis, M. J., Hoke, A., Haughey, N. J., Rothstein, J. D. & Morrison, B. M., Jan 1 2019, (Accepted/In press) In : Glia.

Research output: Contribution to journalArticle

Prospective natural history study of C9orf72 ALS clinical characteristics and biomarkers

Alzheimer's Disease Neuroimaging Initiative, Oct 22 2019, In : Neurology. 93, 17, p. e1605-e1617

Research output: Contribution to journalArticle

Shared polygenic risk and causal inferences in amyotrophic lateral sclerosis

The ITALSGEN Consortium & The International ALS Genomics Consortium, Apr 1 2019, In : Annals of neurology. 85, 4, p. 470-481 12 p.

Research output: Contribution to journalArticle

The role of mutations associated with familial neurodegenerative disorders on blood-brain barrier function in an iPSC model

Katt, M. E., Mayo, L. N., Ellis, S. E., Machairaki, V., Rothstein, J. D., Cheng, L. & Searson, P. C., Jul 15 2019, In : Fluids and Barriers of the CNS. 16, 1, 20.

Research output: Contribution to journalArticle

Open Access
2018

ALSUntangled 41: “Eric Is Winning”

THE ALSUNTANGLED GROUP, Jan 2 2018, In : Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 19, 1-2, p. 157-160 4 p.

Research output: Contribution to journalArticle

ALSUntangled 43: copper

THE ALSUNTANGLED GROUP, Jul 3 2018, In : Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 19, 5-6, p. 472-476 5 p.

Research output: Contribution to journalArticle

Cortical astroglia undergo transcriptomic dysregulation in the G93A SOD1 ALS mouse model

Miller, S. J., Glatzer, J. C., Hsieh, Y. C. & Rothstein, J. D., Jan 1 2018, (Accepted/In press) In : Journal of Neurogenetics.

Research output: Contribution to journalArticle

Genome-wide Analyses Identify KIF5A as a Novel ALS Gene

ITALSGEN Consortium, Genomic Translation for ALS Care (GTAC) Consortium, ALS Sequencing Consortium, NYGC ALS Consortium, Answer ALS Foundation, Clinical Research in ALS and Related Disorders for Therapeutic Development (CReATe) Consortium, SLAGEN Consortium, French ALS Consortium & Project MinE ALS Sequencing Consortium, Mar 21 2018, In : Neuron. 97, 6, p. 1268-1283.e6

Research output: Contribution to journalArticle

Loss of cerebellar glutamate transporters EAAT4 and GLAST differentially affects the spontaneous firing pattern and survival of Purkinje cells

Perkins, E. M., Clarkson, Y. L., Suminaite, D., Lyndon, A. R., Tanaka, K., Rothstein, J. D., Skehel, P. A., Wyllie, D. J. A. & Jackson, M., Jan 1 2018, In : Human molecular genetics. 27, 15, p. 2614-2627 14 p.

Research output: Contribution to journalArticle

Motor neuron-derived microRNAs cause astrocyte dysfunction in amyotrophic lateral sclerosis

Hoye, M. L., Regan, M. R., Jensen, L. A., Lake, A. M., Reddy, L. V., Vidensky, S., Richard, J-P., Maragakis, N. J., Rothstein, J. D., Dougherty, J. D. & Miller, T. M., Sep 1 2018, In : Brain : a journal of neurology. 141, 9, p. 2561-2575 15 p.

Research output: Contribution to journalArticle

Stress Granule Assembly Disrupts Nucleocytoplasmic Transport

Zhang, K., Daigle, J. G., Cunningham, K. M., Coyne, A. N., Ruan, K., Grima, J. C., Bowen, K. E., Wadhwa, H., Yang, P., Rigo, F., Taylor, J. P., Gitler, A. D., Rothstein, J. D. & Lloyd, T., Jan 1 2018, (Accepted/In press) In : Cell.

Research output: Contribution to journalArticle

Tau Protein Disrupts Nucleocytoplasmic Transport in Alzheimer's Disease

Eftekharzadeh, B., Daigle, J. G., Kapinos, L. E., Coyne, A., Schiantarelli, J., Carlomagno, Y., Cook, C., Miller, S. J., Dujardin, S., Amaral, A. S., Grima, J. C., Bennett, R. E., Tepper, K., DeTure, M., Vanderburgh, C. R., Corjuc, B. T., DeVos, S. L., Gonzalez, J. A., Chew, J., Vidensky, S. & 10 others, Gage, F. H., Mertens, J., Troncoso, J. C., Mandelkow, E., Salvatella, X., Lim, R. Y. H., Petrucelli, L., Wegmann, S., Rothstein, J. D. & Hyman, B. T., Sep 5 2018, In : Neuron. 99, 5, p. 925-940.e7

Research output: Contribution to journalArticle

2017

ALSUntangled 38: L-serine

THE ALSUNTANGLED GROUP, Jan 2 2017, In : Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 18, 1-2, p. 148-151 4 p.

Research output: Contribution to journalArticle

ALSUntangled No. 36: Accilion

THE ALSUNTANGLED GROUP, Jan 2 2017, In : Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 18, 1-2, p. 142-147 6 p.

Research output: Contribution to journalArticle

ALSUntangled No. 37: Inosine*

THE ALSUNTANGLED GROUP, Apr 3 2017, In : Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 18, 3-4, p. 309-312 4 p.

Research output: Contribution to journalArticle

c9orf72 Disease-Related Foci Are Each Composed of One Mutant Expanded Repeat RNA

Liu, J., Hu, J., Ludlow, A. T., Pham, J. T., Shay, J. W., Rothstein, J. D. & Corey, D. R., Feb 16 2017, In : Cell Chemical Biology. 24, 2, p. 141-148 8 p.

Research output: Contribution to journalArticle

Mutant Huntingtin Disrupts the Nuclear Pore Complex

Grima, J. C., Daigle, J. G., Arbez, N., Cunningham, K. C., Zhang, K., Ochaba, J., Geater, C., Morozko, E., Stocksdale, J., Glatzer, J. C., Pham, J. T., Ahmed, I., Peng, Q., Wadhwa, H., Pletnikova, O., Troncoso, J. C., Duan, W., Snyder, S. H., Ranum, L. P. W., Thompson, L. M. & 3 others, Lloyd, T., Ross, C. A. & Rothstein, J. D., Apr 5 2017, In : Neuron. 94, 1, p. 93-107.e6

Research output: Contribution to journalArticle

Phosphorylated neurofilament heavy chain: A biomarker of survival for C9ORF72-associated amyotrophic lateral sclerosis

C9ORF72 Neurofilament Study Group, Jul 2017, In : Annals of neurology. 82, 1, p. 139-146 8 p.

Research output: Contribution to journalArticle

Poly(GP) proteins are a useful pharmacodynamic marker for C9ORF72-associated amyotrophic lateral sclerosis

Gendron, T. F., Chew, J., Stankowski, J. N., Hayes, L. R., Zhang, Y. J., Prudencio, M., Carlomagno, Y., Daughrity, L. M., Jansen-West, K., Perkerson, E. A., O'Raw, A., Cook, C., Pregent, L., Belzil, V., Van Blitterswijk, M., Tabassian, L. J., Lee, C. W., Yue, M., Tong, J., Song, Y. & 59 others, Castanedes-Casey, M., Rousseau, L., Phillips, V., Dickson, D. W., Rademakers, R., Fryer, J. D., Rush, B. K., Pedraza, O., Caputo, A. M., Desaro, P., Palmucci, C., Robertson, A., Heckman, M. G., Diehl, N. N., Wiggs, E., Tierney, M., Braun, L., Farren, J., Lacomis, D., Ladha, S., Fournier, C. N., McCluskey, L. F., Elman, L. B., Toledo, J. B., McBride, J. D., Tiloca, C., Morelli, C., Poletti, B., Solca, F., Prelle, A., Wuu, J., Jockel-Balsarotti, J., Rigo, F., Ambrose, C., Datta, A., Yang, W., Raitcheva, D., Antognetti, G., McCampbell, A., Van Swieten, J. C., Miller, B. L., Boxer, A. L., Brown, R. H., Bowser, R., Miller, T. M., Trojanowski, J. Q., Grossman, M., Berry, J. D., Hu, W. T., Ratti, A., Traynor, B. J., Disney, M. D., Benatar, M., Silani, V., Glass, J. D., Floeter, M. K., Rothstein, J. D., Boylan, K. B. & Petrucelli, L., Mar 29 2017, In : Science translational medicine. 9, 383, eaai7866.

Research output: Contribution to journalArticle

2016

ALSUntangled No. 34: GM604

THE ALSUNTANGLED GROUP, Nov 16 2016, In : Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 17, 7-8, p. 617-621 5 p.

Research output: Contribution to journalArticle

ALSUntangled No. 35: Hyperbaric Oxygen Therapy*

THE ALSUNTANGLED GROUP, Nov 16 2016, In : Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 17, 7-8, p. 622-624 3 p.

Research output: Contribution to journalArticle

Astroglia in thick tissue with super resolution and cellular reconstruction

Miller, S. J. & Rothstein, J. D., Aug 1 2016, In : PLoS One. 11, 8, e0160391.

Research output: Contribution to journalArticle

Astroglial transcriptome dysregulation in early disease of an ALS mutant SOD1 mouse model

Miller, S. J., Zhang, P., Glatzer, J. & Rothstein, J. D., Nov 30 2016, (Accepted/In press) In : Journal of Neurogenetics. p. 1-12 12 p.

Research output: Contribution to journalArticle

C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins

Zhang, Y. J., Gendron, T. F., Grima, J. C., Sasaguri, H., Jansen-West, K., Xu, Y. F., Katzman, R. B., Gass, J., Murray, M. E., Shinohara, M., Lin, W. L., Garrett, A., Stankowski, J. N., Daughrity, L., Tong, J., Perkerson, E. A., Yue, M., Chew, J., Castanedes-Casey, M., Kurti, A. & 16 others, Wang, Z. S., Liesinger, A. M., Baker, J. D., Jiang, J., Lagier-Tourenne, C., Edbauer, D., Cleveland, D. W., Rademakers, R., Boylan, K. B., Bu, G., Link, C. D., Dickey, C. A., Rothstein, J. D., Dickson, D. W., Fryer, J. D. & Petrucelli, L., May 1 2016, In : Nature neuroscience. 19, 5, p. 668-677 10 p.

Research output: Contribution to journalArticle

Generation of GFAP::GFP astrocyte reporter lines from human adult fibroblast-derived iPS cells using zinc-finger nuclease technology

Zhang, P. W., Haidet-Phillips, A. M., Pham, J. T., Lee, Y., Huo, Y., Tienari, P. J., Maragakis, N. J., Sattler, R. & Rothstein, J. D., Jan 1 2016, In : Glia. 64, 1, p. 63-75 13 p.

Research output: Contribution to journalArticle

Motor neuron disease, TDP-43 pathology, and memory deficits in mice expressing ALS-FTD-linked UBQLN2 mutations

Le, N. T. T., Chang, L., Kovlyagina, I., Georgiou, P., Safren, N., Braunstein, K. E., Kvarta, M. D., Dyke, A. M. V., Legates, T. A., Philips, T., Morrison, B., Thompson, S. M., Puche, A. C., Gould, T. D., Rothstein, J. D., Wong, P. C. & Monteiro, M. J., Nov 22 2016, In : Proceedings of the National Academy of Sciences of the United States of America. 113, 47, p. E7580-E7589

Research output: Contribution to journalArticle

Posterior cerebellar Purkinje cells in an SCA5/SPARCA1 mouse model are especially vulnerable to the synergistic effect of loss of β-III spectrin and GLAST

Perkins, E. M., Suminaite, D., Clarkson, Y. L., Lee, S. K., Lyndon, A. R., Rothstein, J. D., Wyllie, D. J. A., Tanaka, K. & Jackson, M., 2016, In : Human Molecular Genetics. 25, 20, p. 4448-4461 14 p.

Research output: Contribution to journalArticle

Stereotyped spatial patterns of functional synaptic connectivity in the cerebellar cortex

Valera, A. M., Binda, F., Pawlowski, S. A., Dupont, J. L., Casella, J. F., Rothstein, J. D., Poulain, B. & Isope, P., Mar 16 2016, In : eLife. 5, MARCH2016, e09862.

Research output: Contribution to journalArticle

The astrocytic transporter SLC7A10 (Asc-1) mediates glycinergic inhibition of spinal cord motor neurons

Ehmsen, J. T., Liu, Y., Wang, Y., Paladugu, N., Johnson, A. E., Rothstein, J. D., du Lac, S., Mattson, M. P. & Hoke, A., Oct 19 2016, In : Scientific Reports. 6, 35592.

Research output: Contribution to journalArticle

The transcription factor Pax6 contributes to the induction of GLT-1 expression in astrocytes through an interaction with a distal enhancer element

Ghosh, M., Lane, M., Krizman, E., Sattler, R., Rothstein, J. D. & Robinson, M. B., Jan 1 2016, In : Journal of Neurochemistry. 136, 2, p. 262-275 14 p.

Research output: Contribution to journalArticle

2015

A comprehensive library of familial human amyotrophic lateral sclerosis induced pluripotent stem cells

Li, Y., Balasubramanian, U., Cohen, D., Zhang, P. W., Mosmiller, E., Sattler, R., Maragakis, N. J. & Rothstein, J. D., Mar 11 2015, In : PloS one. 10, 3, e0118266.

Research output: Contribution to journalArticle

ALSUntangled No. 29: MitoQ

The Alsuntangled Lsuntangled Grouproup, Aug 27 2015, In : Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 16, 5-6, p. 427-429 3 p.

Research output: Contribution to journalArticle

Concordant but Varied Phenotypes among Duchenne Muscular Dystrophy Patient-Specific Myoblasts Derived using a Human iPSC-Based Model

Choi, I. Y., Lim, H. T., Estrellas, K., Mula, J., Cohen, T. V., Zhang, Y., Donnelly, C. J., Richard, J-P., Kim, Y. J., Kim, H., Kazuki, Y., Oshimura, M., Li, H. L., Hotta, A., Rothstein, J. D., Maragakis, N. J., Wagner, K. R. & Lee, G., Sep 9 2015, (Accepted/In press) In : Cell Reports.

Research output: Contribution to journalArticle

Human endogenous retrovirus-K contributes to motor neuron disease

Li, W., Lee, M. H., Henderson, L., Tyagi, R., Bachani, M., Steiner, J., Campanac, E., Hoffman, D. A., Geldern, G. V., Johnson, K., Maric, D., Morris, H. D., Lentz, M., Pak, K., Mammen, A., Ostrow, L., Rothstein, J. & Nath, A., Sep 30 2015, In : Science translational medicine. 7, 307

Research output: Contribution to journalArticle

Rodent models of amyotrophic lateral sclerosis

Philips, T. & Rothstein, J. D., Jan 1 2015, In : Current Protocols in Pharmacology. 2015, p. 5.67.1-5.67.21

Research output: Contribution to journalArticle

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport

Zhang, K., Donnelly, C. J., Haeusler, A. R., Grima, J. C., Machamer, J. B., Steinwald, P., Daley, E. L., Miller, S. J., Cunningham, K. M., Vidensky, S., Gupta, S., Thomas, M. A., Hong, I., Chiu, S. L., Huganir, R. L., Ostrow, L. W., Matunis, M. J., Wang, J., Sattler, R., Lloyd, T. E. & 1 others, Rothstein, J. D., Sep 3 2015, In : Nature. 525, 7567, p. 56-61 6 p.

Research output: Contribution to journalArticle

Transplanted glial restricted precursor cells improve neurobehavioral and neuropathological outcomes in a mouse model of neonatal white matter injury despite limited cell survival

Porambo, M., Phillips, A. W., Marx, J., Ternes, K., Arauz, E., Pletnikov, M., Wilson, M. A., Rothstein, J. D., Johnston, M. V. & Fatemi, A., Mar 1 2015, In : Glia. 63, 3, p. 452-465 14 p.

Research output: Contribution to journalArticle

2014

A selective thyroid hormone β receptor agonist enhances human and rodent oligodendrocyte differentiation

Baxi, E. G., Schott, J. T., Fairchild, A. N., Kirby, L. A., Karani, R., Uapinyoying, P., Pardo-Villamizar, C., Rothstein, J. R., Bergles, D. E. & Calabresi, P. A., Sep 2014, In : Glia. 62, 9, p. 1513-1529 17 p.

Research output: Contribution to journalArticle

C9orf72 nucleotide repeat structures initiate molecular cascades of disease

Haeusler, A. R., Donnelly, C. J., Periz, G., Simko, E. A. J., Shaw, P. G., Kim, M. S., Maragakis, N. J., Troncoso, J. C., Pandey, A., Sattler, R., Rothstein, J. D. & Wang, J., Jan 1 2014, In : Nature. 507, 7491, p. 195-200 6 p.

Research output: Contribution to journalArticle

Discovery of a Biomarker and Lead Small Molecules to Target r(GGGGCC)-Associated Defects in c9FTD/ALS

Su, Z., Zhang, Y., Gendron, T. F., Bauer, P. O., Chew, J., Yang, W. Y., Fostvedt, E., Jansen-West, K., Belzil, V. V., Desaro, P., Johnston, A., Overstreet, K., Oh, S. Y., Todd, P. K., Berry, J. D., Cudkowicz, M. E., Boeve, B. F., Dickson, D., Floeter, M. K., Traynor, B. J. & 9 others, Morelli, C., Ratti, A., Silani, V., Rademakers, R., Brown, R. H., Rothstein, J. D., Boylan, K. B., Petrucelli, L. & Disney, M. D., Sep 3 2014, In : Neuron. 83, 5, p. 1043-1050 8 p.

Research output: Contribution to journalArticle

Efficacy of local polymer-based and systemic delivery of the anti-glutamatergic agents riluzole and memantine in rat glioma models: Laboratory investigation

Yohay, K., Tyler, B., Weaver, K. D., Pardo, A. C., Gincel, D., Blakeley, J., Brem, H. & Rothstein, J. D., Apr 2014, In : Journal of neurosurgery. 120, 4, p. 854-863 10 p.

Research output: Contribution to journalArticle