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Items: 1 to 50 of 118

1.

Repeat-associated non-AUG (RAN) translation mechanisms are running into focus for GGGGCC-repeat associated ALS/FTD.

Goodman LD, Bonini NM.

Prog Neurobiol. 2019 Sep 21:101697. doi: 10.1016/j.pneurobio.2019.101697. [Epub ahead of print] Review.

PMID:
31550516
2.

Poly(ADP-Ribosylation) in Age-Related Neurological Disease.

McGurk L, Rifai OM, Bonini NM.

Trends Genet. 2019 Aug;35(8):601-613. doi: 10.1016/j.tig.2019.05.004. Epub 2019 Jun 7. Review.

PMID:
31182245
3.

Toxic expanded GGGGCC repeat transcription is mediated by the PAF1 complex in C9orf72-associated FTD.

Goodman LD, Prudencio M, Kramer NJ, Martinez-Ramirez LF, Srinivasan AR, Lan M, Parisi MJ, Zhu Y, Chew J, Cook CN, Berson A, Gitler AD, Petrucelli L, Bonini NM.

Nat Neurosci. 2019 Jun;22(6):863-874. doi: 10.1038/s41593-019-0396-1. Epub 2019 May 20.

PMID:
31110321
4.

Drosophila Ref1/ALYREF regulates transcription and toxicity associated with ALS/FTD disease etiologies.

Berson A, Goodman LD, Sartoris AN, Otte CG, Aykit JA, Lee VM, Trojanowski JQ, Bonini NM.

Acta Neuropathol Commun. 2019 Apr 29;7(1):65. doi: 10.1186/s40478-019-0710-x.

5.

eIF4B and eIF4H mediate GR production from expanded G4C2 in a Drosophila model for C9orf72-associated ALS.

Goodman LD, Prudencio M, Srinivasan AR, Rifai OM, Lee VM, Petrucelli L, Bonini NM.

Acta Neuropathol Commun. 2019 Apr 25;7(1):62. doi: 10.1186/s40478-019-0711-9.

6.

Author Correction: Aberrant activation of non-coding RNA targets of transcriptional elongation complexes contributes to TDP-43 toxicity.

Chung CY, Berson A, Kennerdell JR, Sartoris A, Unger T, Porta S, Kim HJ, Smith ER, Shilatifard A, Van Deerlin V, Lee VM, Chen-Plotkin A, Bonini NM.

Nat Commun. 2019 Jan 29;10(1):555. doi: 10.1038/s41467-019-08562-x.

7.

Poly(ADP-ribose) Engages the TDP-43 Nuclear-Localization Sequence to Regulate Granulo-Filamentous Aggregation.

McGurk L, Gomes E, Guo L, Shorter J, Bonini NM.

Biochemistry. 2018 Dec 26;57(51):6923-6926. doi: 10.1021/acs.biochem.8b00910. Epub 2018 Dec 17.

PMID:
30540446
8.

En Masse Analysis of Genetic Modifiers Informs Players and Processes in ALS.

Bonini NM.

Neuroscience. 2019 Jan 1;396:A1-A2. doi: 10.1016/j.neuroscience.2018.11.023. Epub 2018 Nov 23. No abstract available.

PMID:
30476526
9.

Aberrant activation of non-coding RNA targets of transcriptional elongation complexes contributes to TDP-43 toxicity.

Chung CY, Berson A, Kennerdell JR, Sartoris A, Unger T, Porta S, Kim HJ, Smith ER, Shilatifard A, Van Deerlin V, Lee VM, Chen-Plotkin A, Bonini NM.

Nat Commun. 2018 Oct 23;9(1):4406. doi: 10.1038/s41467-018-06543-0. Erratum in: Nat Commun. 2019 Jan 29;10(1):555.

10.

MiR-34 inhibits polycomb repressive complex 2 to modulate chaperone expression and promote healthy brain aging.

Kennerdell JR, Liu N, Bonini NM.

Nat Commun. 2018 Oct 10;9(1):4188. doi: 10.1038/s41467-018-06592-5.

11.

Nuclear poly(ADP-ribose) activity is a therapeutic target in amyotrophic lateral sclerosis.

McGurk L, Mojsilovic-Petrovic J, Van Deerlin VM, Shorter J, Kalb RG, Lee VM, Trojanowski JQ, Lee EB, Bonini NM.

Acta Neuropathol Commun. 2018 Aug 29;6(1):84. doi: 10.1186/s40478-018-0586-1.

12.

Poly(ADP-Ribose) Prevents Pathological Phase Separation of TDP-43 by Promoting Liquid Demixing and Stress Granule Localization.

McGurk L, Gomes E, Guo L, Mojsilovic-Petrovic J, Tran V, Kalb RG, Shorter J, Bonini NM.

Mol Cell. 2018 Sep 6;71(5):703-717.e9. doi: 10.1016/j.molcel.2018.07.002. Epub 2018 Aug 9.

13.

Dipeptide repeat proteins activate a heat shock response found in C9ORF72-ALS/FTLD patients.

Mordes DA, Prudencio M, Goodman LD, Klim JR, Moccia R, Limone F, Pietilainen O, Chowdhary K, Dickson DW, Rademakers R, Bonini NM, Petrucelli L, Eggan K.

Acta Neuropathol Commun. 2018 Jul 4;6(1):55. doi: 10.1186/s40478-018-0555-8.

14.

Epigenetic Regulation in Neurodegenerative Diseases.

Berson A, Nativio R, Berger SL, Bonini NM.

Trends Neurosci. 2018 Sep;41(9):587-598. doi: 10.1016/j.tins.2018.05.005. Epub 2018 Jun 7. Review.

15.

Publisher Correction: Dysregulation of the epigenetic landscape of normal aging in Alzheimer's disease.

Nativio R, Donahue G, Berson A, Lan Y, Amlie-Wolf A, Tuzer F, Toledo JB, Gosai SJ, Gregory BD, Torres C, Trojanowski JQ, Wang LS, Johnson FB, Bonini NM, Berger SL.

Nat Neurosci. 2018 Jul;21(7):1018. doi: 10.1038/s41593-018-0124-2.

PMID:
29556027
16.

Dysregulation of the epigenetic landscape of normal aging in Alzheimer's disease.

Nativio R, Donahue G, Berson A, Lan Y, Amlie-Wolf A, Tuzer F, Toledo JB, Gosai SJ, Gregory BD, Torres C, Trojanowski JQ, Wang LS, Johnson FB, Bonini NM, Berger SL.

Nat Neurosci. 2018 Apr;21(4):497-505. doi: 10.1038/s41593-018-0101-9. Epub 2018 Mar 5. Erratum in: Nat Neurosci. 2018 Mar 19;:.

17.

TDP-43 Promotes Neurodegeneration by Impairing Chromatin Remodeling.

Berson A, Sartoris A, Nativio R, Van Deerlin V, Toledo JB, Porta S, Liu S, Chung CY, Garcia BA, Lee VM, Trojanowski JQ, Johnson FB, Berger SL, Bonini NM.

Curr Biol. 2017 Dec 4;27(23):3579-3590.e6. doi: 10.1016/j.cub.2017.10.024. Epub 2017 Nov 16.

18.

Sedimentation Velocity Analysis with Fluorescence Detection of Mutant Huntingtin Exon 1 Aggregation in Drosophila melanogaster and Caenorhabditis elegans.

Kim SA, D'Acunto VF, Kokona B, Hofmann J, Cunningham NR, Bistline EM, Garcia FJ, Akhtar NM, Hoffman SH, Doshi SH, Ulrich KM, Jones NM, Bonini NM, Roberts CM, Link CD, Laue TM, Fairman R.

Biochemistry. 2017 Sep 5;56(35):4676-4688. doi: 10.1021/acs.biochem.7b00518. Epub 2017 Aug 24.

19.

Editorial overview: Molecular & genetic basis of disease.

Bonini NM, Lee EB, Wasco W, Roses AD.

Curr Opin Genet Dev. 2017 Jun;44:iv-vi. doi: 10.1016/j.gde.2017.04.003. Epub 2017 May 2. No abstract available.

PMID:
28473186
20.

The Sustained Impact of Model Organisms-in Genetics and Epigenetics.

Bonini NM, Berger SL.

Genetics. 2017 Jan;205(1):1-4. doi: 10.1534/genetics.116.187864. No abstract available.

21.

ATXN2 trinucleotide repeat length correlates with risk of ALS.

Sproviero W, Shatunov A, Stahl D, Shoai M, van Rheenen W, Jones AR, Al-Sarraj S, Andersen PM, Bonini NM, Conforti FL, Van Damme P, Daoud H, Del Mar Amador M, Fogh I, Forzan M, Gaastra B, Gellera C, Gitler AD, Hardy J, Fratta P, La Bella V, Le Ber I, Van Langenhove T, Lattante S, Lee YC, Malaspina A, Meininger V, Millecamps S, Orrell R, Rademakers R, Robberecht W, Rouleau G, Ross OA, Salachas F, Sidle K, Smith BN, Soong BW, Sorar├╣ G, Stevanin G, Kabashi E, Troakes C, van Broeckhoven C, Veldink JH, van den Berg LH, Shaw CE, Powell JF, Al-Chalabi A.

Neurobiol Aging. 2017 Mar;51:178.e1-178.e9. doi: 10.1016/j.neurobiolaging.2016.11.010. Epub 2016 Nov 24.

22.

Changes in the Transcriptome of Human Astrocytes Accompanying Oxidative Stress-Induced Senescence.

Crowe EP, Tuzer F, Gregory BD, Donahue G, Gosai SJ, Cohen J, Leung YY, Yetkin E, Nativio R, Wang LS, Sell C, Bonini NM, Berger SL, Johnson FB, Torres C.

Front Aging Neurosci. 2016 Aug 31;8:208. doi: 10.3389/fnagi.2016.00208. eCollection 2016.

23.

Spt4 selectively regulates the expression of C9orf72 sense and antisense mutant transcripts.

Kramer NJ, Carlomagno Y, Zhang YJ, Almeida S, Cook CN, Gendron TF, Prudencio M, Van Blitterswijk M, Belzil V, Couthouis J, Paul JW 3rd, Goodman LD, Daughrity L, Chew J, Garrett A, Pregent L, Jansen-West K, Tabassian LJ, Rademakers R, Boylan K, Graff-Radford NR, Josephs KA, Parisi JE, Knopman DS, Petersen RC, Boeve BF, Deng N, Feng Y, Cheng TH, Dickson DW, Cohen SN, Bonini NM, Link CD, Gao FB, Petrucelli L, Gitler AD.

Science. 2016 Aug 12;353(6300):708-12. doi: 10.1126/science.aaf7791.

24.

Fruit flies on the front line: the translational impact of Drosophila.

Perrimon N, Bonini NM, Dhillon P.

Dis Model Mech. 2016 Mar;9(3):229-31. doi: 10.1242/dmm.024810.

25.

GGGGCC microsatellite RNA is neuritically localized, induces branching defects, and perturbs transport granule function.

Burguete AS, Almeida S, Gao FB, Kalb R, Akins MR, Bonini NM.

Elife. 2015 Dec 9;4:e08881. doi: 10.7554/eLife.08881.

26.

Drosophila as an In Vivo Model for Human Neurodegenerative Disease.

McGurk L, Berson A, Bonini NM.

Genetics. 2015 Oct;201(2):377-402. doi: 10.1534/genetics.115.179457. Review.

27.

Hope on the (fruit) fly: the Drosophila wing paradigm of axon injury.

Fang Y, Bonini NM.

Neural Regen Res. 2015 Feb;10(2):173-5. doi: 10.4103/1673-5374.152359. No abstract available.

28.

The exonuclease Nibbler regulates age-associated traits and modulates piRNA length in Drosophila.

Feltzin VL, Khaladkar M, Abe M, Parisi M, Hendriks GJ, Kim J, Bonini NM.

Aging Cell. 2015 Jun;14(3):443-52. doi: 10.1111/acel.12323. Epub 2015 Mar 6.

29.

Ataxin-2 expands insight into the ALS clinical spectrum.

Bonini NM, Hardiman O.

Neurology. 2015 Jan 20;84(3):224-5. doi: 10.1212/WNL.0000000000001167. Epub 2014 Dec 19. No abstract available.

PMID:
25527266
30.

Age-dependent patterns of microRNA RISC loading.

Grigoriev A, Bonini NM.

Aging (Albany NY). 2014 Sep;6(9):705-6. No abstract available.

31.

A fly model for the CCUG-repeat expansion of myotonic dystrophy type 2 reveals a novel interaction with MBNL1.

Yu Z, Goodman LD, Shieh SY, Min M, Teng X, Zhu Y, Bonini NM.

Hum Mol Genet. 2015 Feb 15;24(4):954-62. doi: 10.1093/hmg/ddu507. Epub 2014 Oct 9.

32.

Axon injury and regeneration in the adult Drosophila.

Soares L, Parisi M, Bonini NM.

Sci Rep. 2014 Aug 27;4:6199. doi: 10.1038/srep06199.

33.

Poly-A binding protein-1 localization to a subset of TDP-43 inclusions in amyotrophic lateral sclerosis occurs more frequently in patients harboring an expansion in C9orf72.

McGurk L, Lee VM, Trojanowksi JQ, Van Deerlin VM, Lee EB, Bonini NM.

J Neuropathol Exp Neurol. 2014 Sep;73(9):837-45. doi: 10.1097/NEN.0000000000000102.

34.

Impact of age-associated increase in 2'-O-methylation of miRNAs on aging and neurodegeneration in Drosophila.

Abe M, Naqvi A, Hendriks GJ, Feltzin V, Zhu Y, Grigoriev A, Bonini NM.

Genes Dev. 2014 Jan 1;28(1):44-57. doi: 10.1101/gad.226654.113.

35.

Therapeutic modulation of eIF2╬▒ phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models.

Kim HJ, Raphael AR, LaDow ES, McGurk L, Weber RA, Trojanowski JQ, Lee VM, Finkbeiner S, Gitler AD, Bonini NM.

Nat Genet. 2014 Feb;46(2):152-60. doi: 10.1038/ng.2853. Epub 2013 Dec 15.

36.

Hsp104 suppresses polyglutamine-induced degeneration post onset in a drosophila MJD/SCA3 model.

Cushman-Nick M, Bonini NM, Shorter J.

PLoS Genet. 2013;9(9):e1003781. doi: 10.1371/journal.pgen.1003781. Epub 2013 Sep 5.

37.
38.

MicroRNAs and neurodegeneration: role and impact.

Abe M, Bonini NM.

Trends Cell Biol. 2013 Jan;23(1):30-6. doi: 10.1016/j.tcb.2012.08.013. Epub 2012 Sep 28. Review.

39.

Axon degeneration and regeneration: insights from Drosophila models of nerve injury.

Fang Y, Bonini NM.

Annu Rev Cell Dev Biol. 2012;28:575-97. doi: 10.1146/annurev-cellbio-101011-155836. Epub 2012 Jul 23. Review.

PMID:
22831639
40.

Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis.

Couthouis J, Hart MP, Erion R, King OD, Diaz Z, Nakaya T, Ibrahim F, Kim HJ, Mojsilovic-Petrovic J, Panossian S, Kim CE, Frackelton EC, Solski JA, Williams KL, Clay-Falcone D, Elman L, McCluskey L, Greene R, Hakonarson H, Kalb RG, Lee VM, Trojanowski JQ, Nicholson GA, Blair IP, Bonini NM, Van Deerlin VM, Mourelatos Z, Shorter J, Gitler AD.

Hum Mol Genet. 2012 Jul 1;21(13):2899-911. doi: 10.1093/hmg/dds116. Epub 2012 Mar 27.

41.

A novel Drosophila model of nerve injury reveals an essential role of Nmnat in maintaining axonal integrity.

Fang Y, Soares L, Teng X, Geary M, Bonini NM.

Curr Biol. 2012 Apr 10;22(7):590-5. doi: 10.1016/j.cub.2012.01.065. Epub 2012 Mar 15.

42.

The microRNA miR-34 modulates ageing and neurodegeneration in Drosophila.

Liu N, Landreh M, Cao K, Abe M, Hendriks GJ, Kennerdell JR, Zhu Y, Wang LS, Bonini NM.

Nature. 2012 Feb 15;482(7386):519-23. doi: 10.1038/nature10810.

43.

Cell biology. Yeast informs Alzheimer's disease.

McGurk L, Bonini NM.

Science. 2011 Dec 2;334(6060):1212-3. doi: 10.1126/science.1216073. No abstract available.

PMID:
22144604
44.

A yeast functional screen predicts new candidate ALS disease genes.

Couthouis J, Hart MP, Shorter J, DeJesus-Hernandez M, Erion R, Oristano R, Liu AX, Ramos D, Jethava N, Hosangadi D, Epstein J, Chiang A, Diaz Z, Nakaya T, Ibrahim F, Kim HJ, Solski JA, Williams KL, Mojsilovic-Petrovic J, Ingre C, Boylan K, Graff-Radford NR, Dickson DW, Clay-Falcone D, Elman L, McCluskey L, Greene R, Kalb RG, Lee VM, Trojanowski JQ, Ludolph A, Robberecht W, Andersen PM, Nicholson GA, Blair IP, King OD, Bonini NM, Van Deerlin V, Rademakers R, Mourelatos Z, Gitler AD.

Proc Natl Acad Sci U S A. 2011 Dec 27;108(52):20881-90. doi: 10.1073/pnas.1109434108. Epub 2011 Nov 7.

45.

The exoribonuclease Nibbler controls 3' end processing of microRNAs in Drosophila.

Liu N, Abe M, Sabin LR, Hendriks GJ, Naqvi AS, Yu Z, Cherry S, Bonini NM.

Curr Biol. 2011 Nov 22;21(22):1888-93. doi: 10.1016/j.cub.2011.10.006. Epub 2011 Nov 3.

46.

Protein interacting with C kinase (PICK1) is a suppressor of spinocerebellar ataxia 3-associated neurodegeneration in Drosophila.

McGurk L, Bonini NM.

Hum Mol Genet. 2012 Jan 1;21(1):76-84. doi: 10.1093/hmg/ddr439. Epub 2011 Sep 23.

47.

Genes and pathways affected by CAG-repeat RNA-based toxicity in Drosophila.

Shieh SY, Bonini NM.

Hum Mol Genet. 2011 Dec 15;20(24):4810-21. doi: 10.1093/hmg/ddr420. Epub 2011 Sep 20.

48.

Modeling human trinucleotide repeat diseases in Drosophila.

Yu Z, Bonini NM.

Int Rev Neurobiol. 2011;99:191-212. doi: 10.1016/B978-0-12-387003-2.00008-2. Review.

PMID:
21906541
49.

Model organisms reveal insight into human neurodegenerative disease: ataxin-2 intermediate-length polyglutamine expansions are a risk factor for ALS.

Bonini NM, Gitler AD.

J Mol Neurosci. 2011 Nov;45(3):676-83. doi: 10.1007/s12031-011-9548-9. Epub 2011 Jun 10. Review.

50.

Evaluating the prevalence of polyglutamine repeat expansions in amyotrophic lateral sclerosis.

Lee T, Li YR, Chesi A, Hart MP, Ramos D, Jethava N, Hosangadi D, Epstein J, Hodges B, Bonini NM, Gitler AD.

Neurology. 2011 Jun 14;76(24):2062-5. doi: 10.1212/WNL.0b013e31821f4447. Epub 2011 May 11.

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