Johns Hopkins School of Medicine

Donnelly CJ, Zhang PW, Pham JT, Heusler AR, Mistry NA, Vidensky S, Daley EL, Poth EM, Hoover B, Fines DM, Maragakis N, Tienari PJ, Petrucelli L, Traynor BJ, Wang J, Rigo F, Bennett CF, Blackshaw S, Sattler R, Rothstein JD. RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention. Neuron. 2013;80(2):415-28. Epub 2013/10/22. doi: 10.1016/j.neuron.2013.10.015. PubMed PMID: 24139042. PMCID: PMC4098943

Haeusler AR, Donnelly CJ, Periz G, Simko EA, Shaw PG, Kim MS, Maragakis NJ, Troncoso JC, Pandey A, Sattler R, Rothstein JD, Wang J. C9orf72 nucleotide repeat structures initiate molecular cascades of disease. Nature. 2014;507(7491):195-200. doi: 10.1038/nature13124. PubMed PMID: 24598541; PMCID: 4046618.

Haeusler AR, Donnelly CJ, Rothstein JD. The expanding biology of the C9orf72 nucleotide repeat expansion in neurodegenerative disease. Nat Rev Neurosci. 2016;17(6):383-95. doi: 10.1038/nrn.2016.38. PubMed PMID: 27150398

Zhang K, Donnelly CJ, Haeusler AR, Grima JC, Machamer JB, Steinwald P, Daley EL, Miller SJ, Cunningham KM, Vidensky S, Gupta S, Thomas MA, Hong I, Chiu SL, Huganir RL, Ostrow LW, Matunis MJ, Wang J, Sattler R, Lloyd TE, Rothstein JD. The C9orf72 repeat expansion disrupts nucleocytoplasmic transport. Nature. 2015;525(7567):56-61. doi: 10.1038/nature14973. PubMed PMID: 26308891, PMCID: PMC4800742

Grima JC, Daigle JG, Arbez N, Cunningham KC, Zhang K, Ochaba J, Geater C, Morozko E, Stocksdale J, Glatzer JC, Pham JT, Ahmed I, Peng Q, Wadhwa H, Pletnikova O, Troncoso JC, Duan W, Snyder SH, Ranum LP, Thompson LM, Lloyd TE, Ross CA, Rothstein JD. Mutant Huntingtin Disrupts the Nuclear Pore Complex. Neuron. 2017;94(1):93-107 e6. doi: 10.1016/j.neuron.2017.03.023. PubMed PMID: 28384479.

Zhang Ke, Daigle JG, Cunningham KM, Coyne AM, Ruan K, Grima JC, Bowen KE, Wadhwa H, Yang P, Rigo F, Taylor JP, Gitler AD, Rothstein JD*, Lloyd TE. Stress granule formation disrupts nucleocytoplasmic transport. Cell. 2018 Apr 19, (in press). *Lead Communicating Author

Rothstein JD, Martin LJ, Kuncl RW. Decreased glutamate transport by the brain and spinal cord in amyotrophic lateral sclerosis. N EnglJ Med. 1992;326(22):1464-8. PubMed PMID: 3562.

Lin CL, Bristol LA, Jin L, Dykes-Hoberg M, Crawford T, Clawson L, Rothstein JD. Aberrant RNA processing in a neurodegenerative disease: the cause for absent EAAT2, a glutamate transporter, in amyotrophic lateral sclerosis. Neuron. 1998;20(3):589-602. PubMed PMID: 3522.

Rothstein JD, Dykes-Hoberg M, Pardo CA, Bristol LA, Jin L, Kuncl RW, Kanai Y, Hediger MA, Wang Y, Schielke JP, Welty DF. Knockout of glutamate transporters reveals a major role for astroglial transport in excitotoxicity and clearance of glutamate. Neuron. 1996;16(3):675-86. PubMed PMID: 3544.

Jackson M, Song W, Liu MY, Jin L, Dykes-Hoberg M, Lin CI, Bowers WJ, Federoff HJ, Sternweis PC, Rothstein JD. Modulation of the neuronal glutamate transporter EAAT4 by two interacting proteins. Nature. 2001;410(6824):89-93. PubMed PMID: 9321.
Ikeda Y, Dick KA, Weatherspoon MR, Gincel D, Armbrust KR, Dalton JC, Stevanin G, Durr A,

Yang Y, Gozen O, Watkins A, Lorenzini I, Lepore A, Gao Y, Vidensky S, Brennan J, Poulsen D, Won Park J, Li Jeon N, Robinson MB, Rothstein JD. Presynaptic regulation of astroglial excitatory neurotransmitter transporter GLT1. Neuron. 2009;61(6):880-94. Epub 2009/03/28. doi: S0896-6273(09)00155-X [pii]10.1016/j.neuron.2009.02.010. PubMed PMID: 19323997. PMCID: PMC2743171

Lee Y, Morrison BM, Li Y, Lengacher S, Farah MH, Hoffman PN, Liu Y, Tsingalia A, Jin L, Zhang PW, Pellerin L, Magistretti PJ, Rothstein JD. Oligodendroglia metabolically support axons and contribute to neurodegeneration. Nature. 2012;487(7408):443-8. Epub 2012/07/18. doi: 10.1038/nature11314. PubMed PMID: 22801498; PMCID: PMC3408792.

Kang SH, Fukaya M, Yang JK, Rothstein JD, Bergles DE. NG2+ CNS glial progenitors remain committed to the oligodendrocyte lineage in postnatal life and following neurodegeneration. Neuron. 2010;68(4):668-81. Epub 2010/11/26. doi: 10.1016/j.neuron.2010.09.009. PubMed PMID: 21092857; PMCID: PMC2989827.

Kang SH, Li Y, Fukaya M, Lorenzini I, Cleveland DW, Ostrow LW, Rothstein JD, Bergles DE. Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis. Nat Neurosci. 2013;16(5):571-9. Epub 2013/04/02. doi: 10.1038/nn.3357. PubMed PMID: 23542689; PMCID: PMC3637847.