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Don Cleveland

TitleProfessor
InstitutionUniversity of California San Diego
DepartmentCellular and Molecular Medicine
Address9500 Gilman Drive #0670
La Jolla CA 92093
Phone858-534-7811
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    Collapse Overview 
    Collapse Overview
    Cleveland is interested in the genes and proteins involved in mitotic spindle assembly and faithful chromosome segregation just prior to division. This process is particularly relevant to the development of cancer because it is thought that cancer cells mis-segregate chromosomes due to errors in mitosis resulting in aneuploidy. The major pathway guarding against whole chromosome mis-segregation is the mitotic checkpoint, a cell cycle control mechanism in which the centromeres of unattached chromosomes generate an inhibitor that blocks cyclin B degradation and advance across mitosis. The antitumor drug taxol is effective by chronically activating this checkpoint. In the past year, starting from all purified components Cleveland’s efforts have reconstructed mitotic checkpoint signaling in vitro. Additionally, abnormalities in number of microtubule organizing centers (centrosomes) can promote errors in spindle formation that lead to subsequent chromosome missegregation and extra centrosomes are associated with many cancers. Like DNA, centrosomes are duplicated only once each cell cycle. The duplication process is governed by polo-like kinase 4 (Plk4). Cleveland’s recent efforts identified how centrosome over-replication is blocked: kinase-mediated, autoregulated instability of Plk4 acts to self-limit Plk4 activity so as to prevent centrosome amplification.

    Clinical Interests
    - Cancer Biology
    - Amyotrophic lateral sclerosis


    Collapse Research 
    Collapse Research Activities and Funding
    Mechanisms of chromosome segregation, aneuploidy, and tumorigenesis
    NIH/NIGMS R35GM122476May 1, 2017 - Feb 28, 2022
    Role: Principal Investigator
    Junior Faculty and Postdoctoral Fellows Career Development Workshop
    NIH/NIGMS R13GM111037Sep 15, 2014 - Aug 31, 2015
    Role: Principal Investigator
    ALS therapies and genomics for mutant TDP-43 and TLS/FUS
    NIH/NINDS RC1NS069144Sep 30, 2009 - Aug 31, 2012
    Role: Principal Investigator
    Delivery of therapeutic genes in motor neuron disease
    NIH/NINDS RC2NS069476Sep 30, 2009 - Aug 31, 2012
    Role: Co-Principal Investigator
    Mammalian Centromere Assembly
    NIH/NIGMS R01GM074150Feb 1, 2006 - Aug 31, 2018
    Role: Principal Investigator
    6TH INTERNATIONAL CONGRESS ON CELL BIOLOGY
    NIH/NCI R13CA072388Sep 30, 1996 - Sep 29, 1997
    Role: Principal Investigator
    Disease mechanism in ALS and frontotemporal dementia
    NIH/NINDS R01NS027036Apr 1, 1989 - Aug 31, 2022
    Role: Principal Investigator
    Neurofilaments, SOD1 and Motor Neuron Diseases
    NIH/NINDS R37NS027036Apr 1, 1989 - May 31, 2012
    Role: Principal Investigator
    MICROTUBULE REGULATION: CONTROL OF EXPRESSION OF TUBULI
    NIH/NICHD K04HD000504Sep 30, 1982 - Aug 31, 1987
    Role: Principal Investigator
    Guarding the Genome: Aneuploidy and tumorigenesis
    NIH/NIGMS R01GM029513Jul 1, 1981 - Aug 31, 2020
    Role: Principal Investigator
    MICROTUBULE REGULATION
    NIH/NIGMS R37GM029513Jul 1, 1981 - Jun 30, 2004
    Role: Principal Investigator

    Collapse ORNG Applications 
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    Collapse Bibliographic 
    Collapse Publications
    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Researchers can login to make corrections and additions, or contact us for help.
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    1. Yu H, Cleveland DW. Tuning Apoptosis and Neuroinflammation: TBK1 Restrains RIPK1. Cell. 2018 Sep 06; 174(6):1339-1341. PMID: 30193106.
      View in: PubMed
    2. Gudimchuk N, Tarasovetc EV, Mustyatsa V, Drobyshev AL, Vitre B, Cleveland DW, Ataullakhanov FI, Grishchuk EL. Probing Mitotic CENP-E Kinesin with the Tethered Cargo Motion Assay and Laser Tweezers. Biophys J. 2018 Jun 05; 114(11):2640-2652. PMID: 29874614.
      View in: PubMed
    3. Bennett CL, Dastidar SG, Ling SC, Malik B, Ashe T, Wadhwa M, Miller DB, Lee C, Mitchell MB, van Es MA, Grunseich C, Chen Y, Sopher BL, Greensmith L, Cleveland DW, La Spada AR. Senataxin mutations elicit motor neuron degeneration phenotypes and yield TDP-43 mislocalization in ALS4 mice and human patients. Acta Neuropathol. 2018 May 03. PMID: 29725819.
      View in: PubMed
    4. McMahon MA, Prakash TP, Cleveland DW, Bennett CF, Rahdar M. Chemically Modified Cpf1-CRISPR RNAs Mediate Efficient Genome Editing in Mammalian Cells. Mol Ther. 2018 May 02; 26(5):1228-1240. PMID: 29650467.
      View in: PubMed
    5. Bakhoum SF, Ngo B, Laughney AM, Cavallo JA, Murphy CJ, Ly P, Shah P, Sriram RK, Watkins TBK, Taunk NK, Duran M, Pauli C, Shaw C, Chadalavada K, Rajasekhar VK, Genovese G, Venkatesan S, Birkbak NJ, McGranahan N, Lundquist M, LaPlant Q, Healey JH, Elemento O, Chung CH, Lee NY, Imielenski M, Nanjangud G, Pe'er D, Cleveland DW, Powell SN, Lammerding J, Swanton C, Cantley LC. Chromosomal instability drives metastasis through a cytosolic DNA response. Nature. 2018 Jan 25; 553(7689):467-472. PMID: 29342134.
      View in: PubMed
    6. Cheng W, Wang S, Mestre AA, Fu C, Makarem A, Xian F, Hayes LR, Lopez-Gonzalez R, Drenner K, Jiang J, Cleveland DW, Sun S. C9ORF72 GGGGCC repeat-associated non-AUG translation is upregulated by stress through eIF2a phosphorylation. Nat Commun. 2018 Jan 04; 9(1):51. PMID: 29302060.
      View in: PubMed
    7. Saberi S, Stauffer JE, Jiang J, Garcia SD, Taylor AE, Schulte D, Ohkubo T, Schloffman CL, Maldonado M, Baughn M, Rodriguez MJ, Pizzo D, Cleveland D, Ravits J. Sense-encoded poly-GR dipeptide repeat proteins correlate to neurodegeneration and uniquely co-localize with TDP-43 in dendrites of repeat-expanded C9orf72 amyotrophic lateral sclerosis. Acta Neuropathol. 2018 Mar; 135(3):459-474. PMID: 29196813.
      View in: PubMed
    8. Gao FB, Richter JD, Cleveland DW. Rethinking Unconventional Translation in Neurodegeneration. Cell. 2017 Nov 16; 171(5):994-1000. PMID: 29149615.
      View in: PubMed
    9. Polymenidou M, Cleveland DW. Biological Spectrum of Amyotrophic Lateral Sclerosis Prions. Cold Spring Harb Perspect Med. 2017 Nov 01; 7(11). PMID: 28062558.
      View in: PubMed
    10. Ly P, Cleveland DW. Rebuilding Chromosomes After Catastrophe: Emerging Mechanisms of Chromothripsis. Trends Cell Biol. 2017 Dec; 27(12):917-930. PMID: 28899600.
      View in: PubMed
    11. Ye Q, Kim DH, Dereli I, Rosenberg SC, Hagemann G, Herzog F, Tóth A, Cleveland DW, Corbett KD. The AAA+ ATPase TRIP13 remodels HORMA domains through N-terminal engagement and unfolding. EMBO J. 2017 08 15; 36(16):2419-2434. PMID: 28659378.
      View in: PubMed
    12. Ly P, Cleveland DW. Interrogating cell division errors using random and chromosome-specific missegregation approaches. Cell Cycle. 2017 Jul 03; 16(13):1252-1258. PMID: 28650219.
      View in: PubMed
    13. Guo LY, Allu PK, Zandarashvili L, McKinley KL, Sekulic N, Dawicki-McKenna JM, Fachinetti D, Logsdon GA, Jamiolkowski RM, Cleveland DW, Cheeseman IM, Black BE. Centromeres are maintained by fastening CENP-A to DNA and directing an arginine anchor-dependent nucleosome transition. Nat Commun. 2017 Jun 09; 8:15775. PMID: 28598437.
      View in: PubMed
    14. Gasset-Rosa F, Chillon-Marinas C, Goginashvili A, Atwal RS, Artates JW, Tabet R, Wheeler VC, Bang AG, Cleveland DW, Lagier-Tourenne C. Polyglutamine-Expanded Huntingtin Exacerbates Age-Related Disruption of Nuclear Integrity and Nucleocytoplasmic Transport. Neuron. 2017 Apr 05; 94(1):48-57.e4. PMID: 28384474.
      View in: PubMed
    15. Ditsworth D, Maldonado M, McAlonis-Downes M, Sun S, Seelman A, Drenner K, Arnold E, Ling SC, Pizzo D, Ravits J, Cleveland DW, Da Cruz S. Mutant TDP-43 within motor neurons drives disease onset but not progression in amyotrophic lateral sclerosis. Acta Neuropathol. 2017 Jun; 133(6):907-922. PMID: 28357566.
      View in: PubMed
    16. Da Cruz S, Bui A, Saberi S, Lee SK, Stauffer J, McAlonis-Downes M, Schulte D, Pizzo DP, Parone PA, Cleveland DW, Ravits J. Misfolded SOD1 is not a primary component of sporadic ALS. Acta Neuropathol. 2017 Jul; 134(1):97-111. PMID: 28247063.
      View in: PubMed
    17. Nechemia-Arbely Y, Fachinetti D, Miga KH, Sekulic N, Soni GV, Kim DH, Wong AK, Lee AY, Nguyen K, Dekker C, Ren B, Black BE, Cleveland DW. Human centromeric CENP-A chromatin is a homotypic, octameric nucleosome at all cell cycle points. J Cell Biol. 2017 03 06; 216(3):607-621. PMID: 28235947.
      View in: PubMed
    18. Levine MS, Bakker B, Boeckx B, Moyett J, Lu J, Vitre B, Spierings DC, Lansdorp PM, Cleveland DW, Lambrechts D, Foijer F, Holland AJ. Centrosome Amplification Is Sufficient to Promote Spontaneous Tumorigenesis in Mammals. Dev Cell. 2017 02 06; 40(3):313-322.e5. PMID: 28132847.
      View in: PubMed
    19. Fachinetti D, Logsdon GA, Abdullah A, Selzer EB, Cleveland DW, Black BE. CENP-A Modifications on Ser68 and Lys124 Are Dispensable for Establishment, Maintenance, and Long-Term Function of Human Centromeres. Dev Cell. 2017 01 09; 40(1):104-113. PMID: 28073008.
      View in: PubMed
    20. Jiang J, Cleveland DW. Bidirectional Transcriptional Inhibition as Therapy for ALS/FTD Caused by Repeat Expansion in C9orf72. Neuron. 2016 Dec 21; 92(6):1160-1163. PMID: 28009271.
      View in: PubMed
    21. McMahon MA, Cleveland DW. Gene therapy: Gene-editing therapy for neurological disease. Nat Rev Neurol. 2017 Jan; 13(1):7-9. PMID: 27982043.
      View in: PubMed
    22. Ly P, Teitz LS, Kim DH, Shoshani O, Skaletsky H, Fachinetti D, Page DC, Cleveland DW. Selective Y centromere inactivation triggers chromosome shattering in micronuclei and repair by non-homologous end joining. Nat Cell Biol. 2017 Jan; 19(1):68-75. PMID: 27918550.
      View in: PubMed
    23. Hoffmann S, Dumont M, Barra V, Ly P, Nechemia-Arbely Y, McMahon MA, Hervé S, Cleveland DW, Fachinetti D. CENP-A Is Dispensable for Mitotic Centromere Function after Initial Centromere/Kinetochore Assembly. Cell Rep. 2016 11 22; 17(9):2394-2404. PMID: 27880912.
      View in: PubMed
    24. Taylor JP, Brown RH, Cleveland DW. Decoding ALS: from genes to mechanism. Nature. 2016 11 10; 539(7628):197-206. PMID: 27830784.
      View in: PubMed
    25. Miyanohara A, Kamizato K, Juhas S, Juhasova J, Navarro M, Marsala S, Lukacova N, Hruska-Plochan M, Curtis E, Gabel B, Ciacci J, Ahrens ET, Kaspar BK, Cleveland D, Marsala M. Potent spinal parenchymal AAV9-mediated gene delivery by subpial injection in adult rats and pigs. Mol Ther Methods Clin Dev. 2016; 3:16046. PMID: 27462649; PMCID: PMC4943453.
    26. Seredenina T, Nayernia Z, Sorce S, Maghzal GJ, Filippova A, Ling SC, Basset O, Plastre O, Daali Y, Rushing EJ, Giordana MT, Cleveland DW, Aguzzi A, Stocker R, Krause KH, Jaquet V. Evaluation of NADPH oxidases as drug targets in a mouse model of familial amyotrophic lateral sclerosis. Free Radic Biol Med. 2016 08; 97:95-108. PMID: 27212019.
      View in: PubMed
    27. Jiang J, Zhu Q, Gendron TF, Saberi S, McAlonis-Downes M, Seelman A, Stauffer JE, Jafar-Nejad P, Drenner K, Schulte D, Chun S, Sun S, Ling SC, Myers B, Engelhardt J, Katz M, Baughn M, Platoshyn O, Marsala M, Watt A, Heyser CJ, Ard MC, De Muynck L, Daughrity LM, Swing DA, Tessarollo L, Jung CJ, Delpoux A, Utzschneider DT, Hedrick SM, de Jong PJ, Edbauer D, Van Damme P, Petrucelli L, Shaw CE, Bennett CF, Da Cruz S, Ravits J, Rigo F, Cleveland DW, Lagier-Tourenne C. Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs. Neuron. 2016 05 04; 90(3):535-50. PMID: 27112497; PMCID: PMC4860075 [Available on 05/04/17].
    28. Zhang YJ, Gendron TF, Grima JC, Sasaguri H, Jansen-West K, Xu YF, Katzman RB, Gass J, Murray ME, Shinohara M, Lin WL, Garrett A, Stankowski JN, Daughrity L, Tong J, Perkerson EA, Yue M, Chew J, Castanedes-Casey M, Kurti A, Wang ZS, Liesinger AM, Baker JD, Jiang J, Lagier-Tourenne C, Edbauer D, Cleveland DW, Rademakers R, Boylan KB, Bu G, Link CD, Dickey CA, Rothstein JD, Dickson DW, Fryer JD, Petrucelli L. C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins. Nat Neurosci. 2016 05; 19(5):668-677. PMID: 26998601.
      View in: PubMed
    29. Da Cruz S, Cleveland DW. CELL BIOLOGY. Disrupted nuclear import-export in neurodegeneration. Science. 2016 Jan 08; 351(6269):125-6. PMID: 26744395; PMCID: PMC4842317.
    30. Quaegebeur A, Segura I, Schmieder R, Verdegem D, Decimo I, Bifari F, Dresselaers T, Eelen G, Ghosh D, Davidson SM, Schoors S, Broekaert D, Cruys B, Govaerts K, De Legher C, Bouché A, Schoonjans L, Ramer MS, Hung G, Bossaert G, Cleveland DW, Himmelreich U, Voets T, Lemmens R, Bennett CF, Robberecht W, De Bock K, Dewerchin M, Ghesquière B, Fendt SM, Carmeliet P. Deletion or Inhibition of the Oxygen Sensor PHD1 Protects against Ischemic Stroke via Reprogramming of Neuronal Metabolism. Cell Metab. 2016 Feb 09; 23(2):280-91. PMID: 26774962; PMCID: PMC4880550 [Available on 02/09/17].
    31. Sun S, Sun Y, Ling SC, Ferraiuolo L, McAlonis-Downes M, Zou Y, Drenner K, Wang Y, Ditsworth D, Tokunaga S, Kopelevich A, Kaspar BK, Lagier-Tourenne C, Cleveland DW. 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 Dec 15; 112(50):E6993-7002. PMID: 26621731; PMCID: PMC4687558.
    32. Rahdar M, McMahon MA, Prakash TP, Swayze EE, Bennett CF, Cleveland DW. Synthetic CRISPR RNA-Cas9-guided genome editing in human cells. Proc Natl Acad Sci U S A. 2015 Dec 22; 112(51):E7110-7. PMID: 26589814; PMCID: PMC4697396.
    33. Kulukian A, Holland AJ, Vitre B, Naik S, Cleveland DW, Fuchs E. Epidermal development, growth control, and homeostasis in the face of centrosome amplification. Proc Natl Acad Sci U S A. 2015 Nov 17; 112(46):E6311-20. PMID: 26578791; PMCID: PMC4655514.
    34. Vitre B, Holland AJ, Kulukian A, Shoshani O, Hirai M, Wang Y, Maldonado M, Cho T, Boubaker J, Swing DA, Tessarollo L, Evans SM, Fuchs E, Cleveland DW. Chronic centrosome amplification without tumorigenesis. Proc Natl Acad Sci U S A. 2015 Nov 17; 112(46):E6321-30. PMID: 26578792; PMCID: PMC4655534.
    35. van Blitterswijk M, Gendron TF, Baker MC, DeJesus-Hernandez M, Finch NA, Brown PH, Daughrity LM, Murray ME, Heckman MG, Jiang J, Lagier-Tourenne C, Edbauer D, Cleveland DW, Josephs KA, Parisi JE, Knopman DS, Petersen RC, Petrucelli L, Boeve BF, Graff-Radford NR, Boylan KB, Dickson DW, Rademakers R. Novel clinical associations with specific C9ORF72 transcripts in patients with repeat expansions in C9ORF72. Acta Neuropathol. 2015 Dec; 130(6):863-76. PMID: 26437865; PMCID: PMC4655160 [Available on 12/01/16].
    36. Gendron TF, van Blitterswijk M, Bieniek KF, Daughrity LM, Jiang J, Rush BK, Pedraza O, Lucas JA, Murray ME, Desaro P, Robertson A, Overstreet K, Thomas CS, Crook JE, Castanedes-Casey M, Rousseau L, Josephs KA, Parisi JE, Knopman DS, Petersen RC, Boeve BF, Graff-Radford NR, Rademakers R, Lagier-Tourenne C, Edbauer D, Cleveland DW, Dickson DW, Petrucelli L, Boylan KB. Cerebellar c9RAN proteins associate with clinical and neuropathological characteristics of C9ORF72 repeat expansion carriers. Acta Neuropathol. 2015 Oct; 130(4):559-73. PMID: 26350237; PMCID: PMC4575385.
    37. Topham C, Tighe A, Ly P, Bennett A, Sloss O, Nelson L, Ridgway RA, Huels D, Littler S, Schandl C, Sun Y, Bechi B, Procter DJ, Sansom OJ, Cleveland DW, Taylor SS. MYC Is a Major Determinant of Mitotic Cell Fate. Cancer Cell. 2015 Jul 13; 28(1):129-40. PMID: 26175417; PMCID: PMC4518499.
    38. Mitchell JC, Constable R, So E, Vance C, Scotter E, Glover L, Hortobagyi T, Arnold ES, Ling SC, McAlonis M, Da Cruz S, Polymenidou M, Tessarolo L, Cleveland DW, Shaw CE. Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS. Acta Neuropathol Commun. 2015 Jun 25; 3:36. PMID: 26108367.
      View in: PubMed
    39. Fachinetti D, Han JS, McMahon MA, Ly P, Abdullah A, Wong AJ, Cleveland DW. DNA Sequence-Specific Binding of CENP-B Enhances the Fidelity of Human Centromere Function. Dev Cell. 2015 May 04; 33(3):314-27. PMID: 25942623; PMCID: PMC4421092.
    40. Bertuzzi S, Cleveland DW. The curious incident of the translational dog that didn't bark. Trends Cell Biol. 2015 Apr; 25(4):187-9. PMID: 25817192; PMCID: PMC4508195.
    41. Holland AJ, Reis RM, Niessen S, Pereira C, Andres DA, Spielmann HP, Cleveland DW, Desai A, Gassmann R. Preventing farnesylation of the dynein adaptor Spindly contributes to the mitotic defects caused by farnesyltransferase inhibitors. Mol Biol Cell. 2015 May 15; 26(10):1845-56. PMID: 25808490; PMCID: PMC4436830.
    42. Israelson A, Ditsworth D, Sun S, Song S, Liang J, Hruska-Plochan M, McAlonis-Downes M, Abu-Hamad S, Zoltsman G, Shani T, Maldonado M, Bui A, Navarro M, Zhou H, Marsala M, Kaspar BK, Da Cruz S, Cleveland DW. Macrophage migration inhibitory factor as a chaperone inhibiting accumulation of misfolded SOD1. Neuron. 2015 Apr 08; 86(1):218-32. PMID: 25801706; PMCID: PMC4393372.
    43. Sun S, Ling SC, Qiu J, Albuquerque CP, Zhou Y, Tokunaga S, Li H, Qiu H, Bui A, Yeo GW, Huang EJ, Eggan K, Zhou H, Fu XD, Lagier-Tourenne C, Cleveland DW. ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP. Nat Commun. 2015 Jan 27; 6:6171. PMID: 25625564.
      View in: PubMed
    44. Han JS, Vitre B, Fachinetti D, Cleveland DW. Bimodal activation of BubR1 by Bub3 sustains mitotic checkpoint signaling. Proc Natl Acad Sci U S A. 2014 Oct 07; 111(40):E4185-93. PMID: 25246557; PMCID: PMC4210015.
    45. Traynor BJ, Cleveland DW. Special Issue on amyotrophic lateral sclerosis. Exp Neurol. 2014 Dec; 262 Pt B:73-4. PMID: 25158310.
      View in: PubMed
    46. Holland AJ, Cleveland DW. Polo-like kinase 4 inhibition: a strategy for cancer therapy? Cancer Cell. 2014 Aug 11; 26(2):151-3. PMID: 25117704; PMCID: PMC4148003.
    47. Bodor DL, Mata JF, Sergeev M, David AF, Salimian KJ, Panchenko T, Cleveland DW, Black BE, Shah JV, Jansen LE. The quantitative architecture of centromeric chromatin. Elife. 2014 Jul 15; 3:e02137. PMID: 25027692; PMCID: PMC4091408.
    48. Vitre B, Gudimchuk N, Borda R, Kim Y, Heuser JE, Cleveland DW, Grishchuk EL. Kinetochore-microtubule attachment throughout mitosis potentiated by the elongated stalk of the kinetochore kinesin CENP-E. Mol Biol Cell. 2014 Aug 01; 25(15):2272-81. PMID: 24920822; PMCID: PMC4116301.
    49. Mirzaa GM, Vitre B, Carpenter G, Abramowicz I, Gleeson JG, Paciorkowski AR, Cleveland DW, Dobyns WB, O'Driscoll M. Mutations in CENPE define a novel kinetochore-centromeric mechanism for microcephalic primordial dwarfism. Hum Genet. 2014 Aug; 133(8):1023-39. PMID: 24748105; PMCID: PMC4415612.
    50. Cortes CJ, Ling SC, Guo LT, Hung G, Tsunemi T, Ly L, Tokunaga S, Lopez E, Sopher BL, Bennett CF, Shelton GD, Cleveland DW, La Spada AR. Muscle expression of mutant androgen receptor accounts for systemic and motor neuron disease phenotypes in spinal and bulbar muscular atrophy. Neuron. 2014 Apr 16; 82(2):295-307. PMID: 24742458.
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    51. Winkler EA, Sengillo JD, Sagare AP, Zhao Z, Ma Q, Zuniga E, Wang Y, Zhong Z, Sullivan JS, Griffin JH, Cleveland DW, Zlokovic BV. Blood-spinal cord barrier disruption contributes to early motor-neuron degeneration in ALS-model mice. Proc Natl Acad Sci U S A. 2014 Mar 18; 111(11):E1035-42. PMID: 24591593.
      View in: PubMed
    52. Crotti A, Benner C, Kerman BE, Gosselin D, Lagier-Tourenne C, Zuccato C, Cattaneo E, Gage FH, Cleveland DW, Glass CK. Mutant Huntingtin promotes autonomous microglia activation via myeloid lineage-determining factors. Nat Neurosci. 2014 Apr; 17(4):513-21. PMID: 24584051.
      View in: PubMed
    53. Lobsiger CS, Cleveland DW. Reply to Woodruff et al.: C1q and C3-dependent complement pathway activation does not contribute to disease in SOD1 mutant ALS mice. Proc Natl Acad Sci U S A. 2014 Jan 07; 111(1):E5. PMID: 24555201.
      View in: PubMed
    54. Meyer K, Ferraiuolo L, Miranda CJ, Likhite S, McElroy S, Renusch S, Ditsworth D, Lagier-Tourenne C, Smith RA, Ravits J, Burghes AH, Shaw PJ, Cleveland DW, Kolb SJ, Kaspar BK. Direct conversion of patient fibroblasts demonstrates non-cell autonomous toxicity of astrocytes to motor neurons in familial and sporadic ALS. Proc Natl Acad Sci U S A. 2014 Jan 14; 111(2):829-32. PMID: 24379375.
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    55. Parone PA, Da Cruz S, Cleveland DW. Mitochondrial Isolation and Purification from Mouse Spinal Cord. Bio Protoc. 2013 Nov 05; 3(21). PMID: 27819013.
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    56. Lobsiger CS, Boillée S, Pozniak C, Khan AM, McAlonis-Downes M, Lewcock JW, Cleveland DW. C1q induction and global complement pathway activation do not contribute to ALS toxicity in mutant SOD1 mice. Proc Natl Acad Sci U S A. 2013 Nov 12; 110(46):E4385-92. PMID: 24170856.
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    57. Lagier-Tourenne C, Baughn M, Rigo F, Sun S, Liu P, Li HR, Jiang J, Watt AT, Chun S, Katz M, Qiu J, Sun Y, Ling SC, Zhu Q, Polymenidou M, Drenner K, Artates JW, McAlonis-Downes M, Markmiller S, Hutt KR, Pizzo DP, Cady J, Harms MB, Baloh RH, Vandenberg SR, Yeo GW, Fu XD, Bennett CF, Cleveland DW, Ravits J. Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration. Proc Natl Acad Sci U S A. 2013 Nov 19; 110(47):E4530-9. PMID: 24170860.
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    58. Silk AD, Zasadil LM, Holland AJ, Vitre B, Cleveland DW, Weaver BA. Chromosome missegregation rate predicts whether aneuploidy will promote or suppress tumors. Proc Natl Acad Sci U S A. 2013 Oct 29; 110(44):E4134-41. PMID: 24133140; PMCID: PMC3816416.
    59. Foust KD, Salazar DL, Likhite S, Ferraiuolo L, Ditsworth D, Ilieva H, Meyer K, Schmelzer L, Braun L, Cleveland DW, Kaspar BK. Therapeutic AAV9-mediated suppression of mutant SOD1 slows disease progression and extends survival in models of inherited ALS. Mol Ther. 2013 Dec; 21(12):2148-59. PMID: 24008656.
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    60. Gudimchuk N, Vitre B, Kim Y, Kiyatkin A, Cleveland DW, Ataullakhanov FI, Grishchuk EL. Kinetochore kinesin CENP-E is a processive bi-directional tracker of dynamic microtubule tips. Nat Cell Biol. 2013 Sep; 15(9):1079-1088. PMID: 23955301; PMCID: PMC3919686.
    61. Ling SC, Polymenidou M, Cleveland DW. Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron. 2013 Aug 07; 79(3):416-38. PMID: 23931993.
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    62. Earnshaw WC, Cleveland DW. CENP-A and the CENP nomenclature: response to Talbert and Henikoff. Trends Genet. 2013 Sep; 29(9):500-2. PMID: 23910159.
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    63. Fachinetti D, Folco HD, Nechemia-Arbely Y, Valente LP, Nguyen K, Wong AJ, Zhu Q, Holland AJ, Desai A, Jansen LE, Cleveland DW. A two-step mechanism for epigenetic specification of centromere identity and function. Nat Cell Biol. 2013 Sep; 15(9):1056-66. PMID: 23873148; PMCID: PMC4418506.
    64. Han JS, Holland AJ, Fachinetti D, Kulukian A, Cetin B, Cleveland DW. Catalytic assembly of the mitotic checkpoint inhibitor BubR1-Cdc20 by a Mad2-induced functional switch in Cdc20. Mol Cell. 2013 Jul 11; 51(1):92-104. PMID: 23791783; PMCID: PMC3713096.
    65. Earnshaw WC, Allshire RC, Black BE, Bloom K, Brinkley BR, Brown W, Cheeseman IM, Choo KH, Copenhaver GP, Deluca JG, Desai A, Diekmann S, Erhardt S, Fitzgerald-Hayes M, Foltz D, Fukagawa T, Gassmann R, Gerlich DW, Glover DM, Gorbsky GJ, Harrison SC, Heun P, Hirota T, Jansen LE, Karpen G, Kops GJ, Lampson MA, Lens SM, Losada A, Luger K, Maiato H, Maddox PS, Margolis RL, Masumoto H, McAinsh AD, Mellone BG, Meraldi P, Musacchio A, Oegema K, O'Neill RJ, Salmon ED, Scott KC, Straight AF, Stukenberg PT, Sullivan BA, Sullivan KF, Sunkel CE, Swedlow JR, Walczak CE, Warburton PE, Westermann S, Willard HF, Wordeman L, Yanagida M, Yen TJ, Yoda K, Cleveland DW. Esperanto for histones: CENP-A, not CenH3, is the centromeric histone H3 variant. Chromosome Res. 2013 Apr; 21(2):101-6. PMID: 23580138.
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    66. 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 May; 16(5):571-9. PMID: 23542689.
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    67. Parone PA, Da Cruz S, Han JS, McAlonis-Downes M, Vetto AP, Lee SK, Tseng E, Cleveland DW. Enhancing mitochondrial calcium buffering capacity reduces aggregation of misfolded SOD1 and motor neuron cell death without extending survival in mouse models of inherited amyotrophic lateral sclerosis. J Neurosci. 2013 Mar 13; 33(11):4657-71. PMID: 23486940.
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    68. Arnold ES, Ling SC, Huelga SC, Lagier-Tourenne C, Polymenidou M, Ditsworth D, Kordasiewicz HB, McAlonis-Downes M, Platoshyn O, Parone PA, Da Cruz S, Clutario KM, Swing D, Tessarollo L, Marsala M, Shaw CE, Yeo GW, Cleveland DW. ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43. Proc Natl Acad Sci U S A. 2013 Feb 19; 110(8):E736-45. PMID: 23382207.
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    69. Holland AJ, Fachinetti D, Zhu Q, Bauer M, Verma IM, Nigg EA, Cleveland DW. The autoregulated instability of Polo-like kinase 4 limits centrosome duplication to once per cell cycle. Genes Dev. 2012 Dec 15; 26(24):2684-9. PMID: 23249732; PMCID: PMC3533073.
    70. Sun S, Cleveland DW. TDP-43 toxicity and the usefulness of junk. Nat Genet. 2012 Dec; 44(12):1289-91. PMID: 23192178.
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    71. Holland AJ, Cleveland DW. The deubiquitinase USP44 is a tumor suppressor that protects against chromosome missegregation. J Clin Invest. 2012 Dec; 122(12):4325-8. PMID: 23187131; PMCID: PMC3533566.
    72. Holland AJ, Fachinetti D, Han JS, Cleveland DW. Inducible, reversible system for the rapid and complete degradation of proteins in mammalian cells. Proc Natl Acad Sci U S A. 2012 Dec 04; 109(49):E3350-7. PMID: 23150568; PMCID: PMC3523849.
    73. Holland AJ, Cleveland DW. Chromoanagenesis and cancer: mechanisms and consequences of localized, complex chromosomal rearrangements. Nat Med. 2012 Nov; 18(11):1630-8. PMID: 23135524; PMCID: PMC3616639.
    74. Vitre BD, Cleveland DW. Centrosomes, chromosome instability (CIN) and aneuploidy. Curr Opin Cell Biol. 2012 Dec; 24(6):809-15. PMID: 23127609; PMCID: PMC3621708.
    75. Lagier-Tourenne C, Polymenidou M, Hutt KR, Vu AQ, Baughn M, Huelga SC, Clutario KM, Ling SC, Liang TY, Mazur C, Wancewicz E, Kim AS, Watt A, Freier S, Hicks GG, Donohue JP, Shiue L, Bennett CF, Ravits J, Cleveland DW, Yeo GW. Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs. Nat Neurosci. 2012 Nov; 15(11):1488-97. PMID: 23023293.
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    76. Yu D, Pendergraff H, Liu J, Kordasiewicz HB, Cleveland DW, Swayze EE, Lima WF, Crooke ST, Prakash TP, Corey DR. Single-stranded RNAs use RNAi to potently and allele-selectively inhibit mutant huntingtin expression. Cell. 2012 Aug 31; 150(5):895-908. PMID: 22939619.
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    77. Hefferan MP, Galik J, Kakinohana O, Sekerkova G, Santucci C, Marsala S, Navarro R, Hruska-Plochan M, Johe K, Feldman E, Cleveland DW, Marsala M. Human neural stem cell replacement therapy for amyotrophic lateral sclerosis by spinal transplantation. PLoS One. 2012; 7(8):e42614. PMID: 22916141.
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    78. Kordasiewicz HB, Stanek LM, Wancewicz EV, Mazur C, McAlonis MM, Pytel KA, Artates JW, Weiss A, Cheng SH, Shihabuddin LS, Hung G, Bennett CF, Cleveland DW. Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis. Neuron. 2012 Jun 21; 74(6):1031-44. PMID: 22726834; PMCID: PMC3383626.
    79. Holland AJ, Cleveland DW. Losing balance: the origin and impact of aneuploidy in cancer. EMBO Rep. 2012 Jun 01; 13(6):501-14. PMID: 22565320; PMCID: PMC3367240.
    80. Polymenidou M, Cleveland DW. Prion-like spread of protein aggregates in neurodegeneration. J Exp Med. 2012 May 07; 209(5):889-93. PMID: 22566400.
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    81. Kolano A, Brunet S, Silk AD, Cleveland DW, Verlhac MH. Error-prone mammalian female meiosis from silencing the spindle assembly checkpoint without normal interkinetochore tension. Proc Natl Acad Sci U S A. 2012 Jul 03; 109(27):E1858-67. PMID: 22552228; PMCID: PMC3390881.
    82. Da Cruz S, Parone PA, Lopes VS, Lillo C, McAlonis-Downes M, Lee SK, Vetto AP, Petrosyan S, Marsala M, Murphy AN, Williams DS, Spiegelman BM, Cleveland DW. Elevated PGC-1a activity sustains mitochondrial biogenesis and muscle function without extending survival in a mouse model of inherited ALS. Cell Metab. 2012 May 02; 15(5):778-86. PMID: 22560226.
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    83. Nechemia-Arbely Y, Fachinetti D, Cleveland DW. Replicating centromeric chromatin: spatial and temporal control of CENP-A assembly. Exp Cell Res. 2012 Jul 15; 318(12):1353-60. PMID: 22561213; PMCID: PMC3616609.
    84. Holland AJ, Fachinetti D, Da Cruz S, Zhu Q, Vitre B, Lince-Faria M, Chen D, Parish N, Verma IM, Bettencourt-Dias M, Cleveland DW. Polo-like kinase 4 controls centriole duplication but does not directly regulate cytokinesis. Mol Biol Cell. 2012 May; 23(10):1838-45. PMID: 22456511; PMCID: PMC3350549.
    85. Polymenidou M, Lagier-Tourenne C, Hutt KR, Bennett CF, Cleveland DW, Yeo GW. Misregulated RNA processing in amyotrophic lateral sclerosis. Brain Res. 2012 Jun 26; 1462:3-15. PMID: 22444279.
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    86. Polymenidou M, Cleveland DW. The seeds of neurodegeneration: prion-like spreading in ALS. Cell. 2011 Oct 28; 147(3):498-508. PMID: 22036560.
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    87. Kolodner RD, Cleveland DW, Putnam CD. Cancer. Aneuploidy drives a mutator phenotype in cancer. Science. 2011 Aug 19; 333(6045):942-3. PMID: 21852477; PMCID: PMC3806716.
    88. Da Cruz S, Cleveland DW. Understanding the role of TDP-43 and FUS/TLS in ALS and beyond. Curr Opin Neurobiol. 2011 Dec; 21(6):904-19. PMID: 21813273.
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    89. Vande Velde C, McDonald KK, Boukhedimi Y, McAlonis-Downes M, Lobsiger CS, Bel Hadj S, Zandona A, Julien JP, Shah SB, Cleveland DW. Misfolded SOD1 associated with motor neuron mitochondria alters mitochondrial shape and distribution prior to clinical onset. PLoS One. 2011; 6(7):e22031. PMID: 21779368.
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    90. Polymenidou M, Lagier-Tourenne C, Hutt KR, Huelga SC, Moran J, Liang TY, Ling SC, Sun E, Wancewicz E, Mazur C, Kordasiewicz H, Sedaghat Y, Donohue JP, Shiue L, Bennett CF, Yeo GW, Cleveland DW. Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43. Nat Neurosci. 2011 Apr; 14(4):459-68. PMID: 21358643.
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    91. Black BE, Cleveland DW. Epigenetic centromere propagation and the nature of CENP-a nucleosomes. Cell. 2011 Feb 18; 144(4):471-9. PMID: 21335232; PMCID: PMC3061232.
    92. Li Q, Vande Velde C, Israelson A, Xie J, Bailey AO, Dong MQ, Chun SJ, Roy T, Winer L, Yates JR, Capaldi RA, Cleveland DW, Miller TM. ALS-linked mutant superoxide dismutase 1 (SOD1) alters mitochondrial protein composition and decreases protein import. Proc Natl Acad Sci U S A. 2010 Dec 07; 107(49):21146-51. PMID: 21078990.
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    93. Hatch EM, Kulukian A, Holland AJ, Cleveland DW, Stearns T. Cep152 interacts with Plk4 and is required for centriole duplication. J Cell Biol. 2010 Nov 15; 191(4):721-9. PMID: 21059850; PMCID: PMC2983069.
    94. Cetin B, Cleveland DW. How to survive aneuploidy. Cell. 2010 Oct 01; 143(1):27-9. PMID: 20887888; PMCID: PMC2955074.
    95. Lan W, Cleveland DW. Multiclassifier proteomics to define complexes yields new chromosomal proteins. Dev Cell. 2010 Sep 14; 19(3):356-9. PMID: 20833356; PMCID: PMC2943763.
    96. Israelson A, Arbel N, Da Cruz S, Ilieva H, Yamanaka K, Shoshan-Barmatz V, Cleveland DW. Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS. Neuron. 2010 Aug 26; 67(4):575-87. PMID: 20797535.
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    97. Lagier-Tourenne C, Cleveland DW. Neurodegeneration: An expansion in ALS genetics. Nature. 2010 Aug 26; 466(7310):1052-3. PMID: 20740002.
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    98. Kim Y, Holland AJ, Lan W, Cleveland DW. Aurora kinases and protein phosphatase 1 mediate chromosome congression through regulation of CENP-E. Cell. 2010 Aug 06; 142(3):444-55. PMID: 20691903; PMCID: PMC2921712.
    99. Holland AJ, Lan W, Cleveland DW. Centriole duplication: A lesson in self-control. Cell Cycle. 2010 Jul 15; 9(14):2731-6. PMID: 20647763; PMCID: PMC3040958.
    100. Lan W, Cleveland DW. A chemical tool box defines mitotic and interphase roles for Mps1 kinase. J Cell Biol. 2010 Jul 12; 190(1):21-4. PMID: 20624898; PMCID: PMC2911672.
    101. Ling SC, Albuquerque CP, Han JS, Lagier-Tourenne C, Tokunaga S, Zhou H, Cleveland DW. 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 Jul 27; 107(30):13318-23. PMID: 20624952.
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    102. Gassmann R, Holland AJ, Varma D, Wan X, Civril F, Cleveland DW, Oegema K, Salmon ED, Desai A. Removal of Spindly from microtubule-attached kinetochores controls spindle checkpoint silencing in human cells. Genes Dev. 2010 May; 24(9):957-71. PMID: 20439434; PMCID: PMC2861194.
    103. Lagier-Tourenne C, Polymenidou M, Cleveland DW. TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration. Hum Mol Genet. 2010 Apr 15; 19(R1):R46-64. PMID: 20400460.
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    104. Radulescu AE, Cleveland DW. NuMA after 30 years: the matrix revisited. Trends Cell Biol. 2010 Apr; 20(4):214-22. PMID: 20137953; PMCID: PMC3137513.
    105. Holland AJ, Lan W, Niessen S, Hoover H, Cleveland DW. Polo-like kinase 4 kinase activity limits centrosome overduplication by autoregulating its own stability. J Cell Biol. 2010 Jan 25; 188(2):191-8. PMID: 20100909; PMCID: PMC2813471.
    106. Gurden MD, Holland AJ, van Zon W, Tighe A, Vergnolle MA, Andres DA, Spielmann HP, Malumbres M, Wolthuis RM, Cleveland DW, Taylor SS. Cdc20 is required for the post-anaphase, KEN-dependent degradation of centromere protein F. J Cell Sci. 2010 Feb 01; 123(Pt 3):321-30. PMID: 20053638; PMCID: PMC2816182.
    107. Ilieva H, Polymenidou M, Cleveland DW. Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond. J Cell Biol. 2009 Dec 14; 187(6):761-72. PMID: 19951898; PMCID: PMC2806318.
    108. Zhong Z, Ilieva H, Hallagan L, Bell R, Singh I, Paquette N, Thiyagarajan M, Deane R, Fernandez JA, Lane S, Zlokovic AB, Liu T, Griffin JH, Chow N, Castellino FJ, Stojanovic K, Cleveland DW, Zlokovic BV. Activated protein C therapy slows ALS-like disease in mice by transcriptionally inhibiting SOD1 in motor neurons and microglia cells. J Clin Invest. 2009 Nov; 119(11):3437-49. PMID: 19841542.
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    109. Zeitlin SG, Baker NM, Chapados BR, Soutoglou E, Wang JY, Berns MW, Cleveland DW. Double-strand DNA breaks recruit the centromeric histone CENP-A. Proc Natl Acad Sci U S A. 2009 Sep 15; 106(37):15762-7. PMID: 19717431; PMCID: PMC2747192.
    110. Samoshkin A, Arnaoutov A, Jansen LE, Ouspenski I, Dye L, Karpova T, McNally J, Dasso M, Cleveland DW, Strunnikov A. Human condensin function is essential for centromeric chromatin assembly and proper sister kinetochore orientation. PLoS One. 2009 Aug 28; 4(8):e6831. PMID: 19714251; PMCID: PMC2730017.
    111. Holland AJ, Cleveland DW. Boveri revisited: chromosomal instability, aneuploidy and tumorigenesis. Nat Rev Mol Cell Biol. 2009 Jul; 10(7):478-87. PMID: 19546858; PMCID: PMC3154738.
    112. Weaver BA, Cleveland DW. The role of aneuploidy in promoting and suppressing tumors. J Cell Biol. 2009 Jun 15; 185(6):935-7. PMID: 19528293; PMCID: PMC2711620.
    113. Foltz DR, Jansen LE, Bailey AO, Yates JR, Bassett EA, Wood S, Black BE, Cleveland DW. Centromere-specific assembly of CENP-a nucleosomes is mediated by HJURP. Cell. 2009 May 01; 137(3):472-84. PMID: 19410544; PMCID: PMC2747366.
    114. Lagier-Tourenne C, Cleveland DW. Rethinking ALS: the FUS about TDP-43. Cell. 2009 Mar 20; 136(6):1001-4. PMID: 19303844.
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    115. Silk AD, Holland AJ, Cleveland DW. Requirements for NuMA in maintenance and establishment of mammalian spindle poles. J Cell Biol. 2009 Mar 09; 184(5):677-90. PMID: 19255246; PMCID: PMC2686415.
    116. Lobsiger CS, Boillee S, McAlonis-Downes M, Khan AM, Feltri ML, Yamanaka K, Cleveland DW. Schwann cells expressing dismutase active mutant SOD1 unexpectedly slow disease progression in ALS mice. Proc Natl Acad Sci U S A. 2009 Mar 17; 106(11):4465-70. PMID: 19251638; PMCID: PMC2657393.
    117. Garcia ML, Rao MV, Fujimoto J, Garcia VB, Shah SB, Crum J, Gotow T, Uchiyama Y, Ellisman M, Calcutt NA, Cleveland DW. Phosphorylation of highly conserved neurofilament medium KSP repeats is not required for myelin-dependent radial axonal growth. J Neurosci. 2009 Feb 04; 29(5):1277-84. PMID: 19193875; PMCID: PMC2782950.
    118. Cleveland DW, Yamanaka K, Bomont P. Gigaxonin controls vimentin organization through a tubulin chaperone-independent pathway. Hum Mol Genet. 2009 Apr 15; 18(8):1384-94. PMID: 19168853; PMCID: PMC2664145.
    119. Kulukian A, Han JS, Cleveland DW. Unattached kinetochores catalyze production of an anaphase inhibitor that requires a Mad2 template to prime Cdc20 for BubR1 binding. Dev Cell. 2009 Jan; 16(1):105-17. PMID: 19154722; PMCID: PMC2655205.
    120. Weaver BA, Cleveland DW. The aneuploidy paradox in cell growth and tumorigenesis. Cancer Cell. 2008 Dec 09; 14(6):431-3. PMID: 19061834; PMCID: PMC3132552.
    121. Ilieva HS, Yamanaka K, Malkmus S, Kakinohana O, Yaksh T, Marsala M, Cleveland DW. Mutant dynein (Loa) triggers proprioceptive axon loss that extends survival only in the SOD1 ALS model with highest motor neuron death. Proc Natl Acad Sci U S A. 2008 Aug 26; 105(34):12599-604. PMID: 18719118; PMCID: PMC2527957.
    122. Holland AJ, Cleveland DW. Beyond genetics: surprising determinants of cell fate in antitumor drugs. Cancer Cell. 2008 Aug 12; 14(2):103-5. PMID: 18691543; PMCID: PMC3132553.
    123. Chang Y, Kong Q, Shan X, Tian G, Ilieva H, Cleveland DW, Rothstein JD, Borchelt DR, Wong PC, Lin CL. Messenger RNA oxidation occurs early in disease pathogenesis and promotes motor neuron degeneration in ALS. PLoS One. 2008 Aug 06; 3(8):e2849. PMID: 18682740; PMCID: PMC2481395.
    124. Dequen F, Bomont P, Gowing G, Cleveland DW, Julien JP. Modest loss of peripheral axons, muscle atrophy and formation of brain inclusions in mice with targeted deletion of gigaxonin exon 1. J Neurochem. 2008 Oct; 107(1):253-64. PMID: 18680552; PMCID: PMC3657508.
    125. Polymenidou M, Cleveland DW. Motor neuron disease: The curious ways of ALS. Nature. 2008 Jul 17; 454(7202):284-5. PMID: 18633404; PMCID: PMC3625042.
    126. Jelluma N, Brenkman AB, McLeod I, Yates JR, Cleveland DW, Medema RH, Kops GJ. Chromosomal instability by inefficient Mps1 auto-activation due to a weakened mitotic checkpoint and lagging chromosomes. PLoS One. 2008 Jun 11; 3(6):e2415. PMID: 18545697; PMCID: PMC2408436.
    127. Yamanaka K, Boillee S, Roberts EA, Garcia ML, McAlonis-Downes M, Mikse OR, Cleveland DW, Goldstein LS. Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice. Proc Natl Acad Sci U S A. 2008 May 27; 105(21):7594-9. PMID: 18492803; PMCID: PMC2396671.
    128. Kim Y, Heuser JE, Waterman CM, Cleveland DW. CENP-E combines a slow, processive motor and a flexible coiled coil to produce an essential motile kinetochore tether. J Cell Biol. 2008 May 05; 181(3):411-9. PMID: 18443223; PMCID: PMC2364708.
    129. Zhong Z, Deane R, Ali Z, Parisi M, Shapovalov Y, O'Banion MK, Stojanovic K, Sagare A, Boillee S, Cleveland DW, Zlokovic BV. ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration. Nat Neurosci. 2008 Apr; 11(4):420-2. PMID: 18344992; PMCID: PMC2895310.
    130. Vande Velde C, Miller TM, Cashman NR, Cleveland DW. Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria. Proc Natl Acad Sci U S A. 2008 Mar 11; 105(10):4022-7. PMID: 18296640; PMCID: PMC2268797.
    131. Yamanaka K, Chun SJ, Boillee S, Fujimori-Tonou N, Yamashita H, Gutmann DH, Takahashi R, Misawa H, Cleveland DW. Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis. Nat Neurosci. 2008 Mar; 11(3):251-3. PMID: 18246065; PMCID: PMC3137510.
    132. Boillée S, Cleveland DW. Revisiting oxidative damage in ALS: microglia, Nox, and mutant SOD1. J Clin Invest. 2008 Feb; 118(2):474-8. PMID: 18219386; PMCID: PMC2213376.
    133. Weaver BA, Silk AD, Cleveland DW. Low rates of aneuploidy promote tumorigenesis while high rates of aneuploidy cause cell death and tumor suppression. Cell Oncol. 2008; 30(5):453. PMID: 18791276; PMCID: PMC4618964.
    134. Weaver BA, Cleveland DW. Aneuploidy: instigator and inhibitor of tumorigenesis. Cancer Res. 2007 Nov 01; 67(21):10103-5. PMID: 17974949; PMCID: PMC3132555.
    135. Lobsiger CS, Cleveland DW. Glial cells as intrinsic components of non-cell-autonomous neurodegenerative disease. Nat Neurosci. 2007 Nov; 10(11):1355-60. PMID: 17965655; PMCID: PMC3110080.
    136. Detmer SA, Vande Velde C, Cleveland DW, Chan DC. Hindlimb gait defects due to motor axon loss and reduced distal muscles in a transgenic mouse model of Charcot-Marie-Tooth type 2A. Hum Mol Genet. 2008 Feb 01; 17(3):367-75. PMID: 17959936.
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    137. Gruzman A, Wood WL, Alpert E, Prasad MD, Miller RG, Rothstein JD, Bowser R, Hamilton R, Wood TD, Cleveland DW, Lingappa VR, Liu J. Common molecular signature in SOD1 for both sporadic and familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A. 2007 Jul 24; 104(30):12524-9. PMID: 17636119; PMCID: PMC1941502.
    138. Bailey AO, Miller TM, Dong MQ, Vande Velde C, Cleveland DW, Yates JR. RCADiA: simple automation platform for comparative multidimensional protein identification technology. Anal Chem. 2007 Aug 15; 79(16):6410-8. PMID: 17616168; PMCID: PMC2528021.
    139. Weaver BA, Cleveland DW. Comment on "A centrosome-independent role for gamma-TuRC proteins in the spindle assembly checkpoint". Science. 2007 May 18; 316(5827):982; author reply 982. PMID: 17510348.
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    140. Rakhit R, Robertson J, Vande Velde C, Horne P, Ruth DM, Griffin J, Cleveland DW, Cashman NR, Chakrabartty A. An immunological epitope selective for pathological monomer-misfolded SOD1 in ALS. Nat Med. 2007 Jun; 13(6):754-9. PMID: 17486090.
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    141. Conde e Silva N, Black BE, Sivolob A, Filipski J, Cleveland DW, Prunell A. CENP-A-containing nucleosomes: easier disassembly versus exclusive centromeric localization. J Mol Biol. 2007 Jul 13; 370(3):555-73. PMID: 17524417.
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    142. Lobsiger CS, Boillée S, Cleveland DW. Toxicity from different SOD1 mutants dysregulates the complement system and the neuronal regenerative response in ALS motor neurons. Proc Natl Acad Sci U S A. 2007 May 01; 104(18):7319-26. PMID: 17463094; PMCID: PMC1863491.
    143. Black BE, Brock MA, Bédard S, Woods VL, Cleveland DW. An epigenetic mark generated by the incorporation of CENP-A into centromeric nucleosomes. Proc Natl Acad Sci U S A. 2007 Mar 20; 104(12):5008-13. PMID: 17360341; PMCID: PMC1829255.
    144. Jansen LE, Black BE, Foltz DR, Cleveland DW. Propagation of centromeric chromatin requires exit from mitosis. J Cell Biol. 2007 Mar 12; 176(6):795-805. PMID: 17339380; PMCID: PMC2064054.
    145. Black BE, Jansen LE, Maddox PS, Foltz DR, Desai AB, Shah JV, Cleveland DW. Centromere identity maintained by nucleosomes assembled with histone H3 containing the CENP-A targeting domain. Mol Cell. 2007 Jan 26; 25(2):309-22. PMID: 17244537.
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    146. Weaver BA, Silk AD, Montagna C, Verdier-Pinard P, Cleveland DW. Aneuploidy acts both oncogenically and as a tumor suppressor. Cancer Cell. 2007 Jan; 11(1):25-36. PMID: 17189716.
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    147. Miller TM, Kim SH, Yamanaka K, Hester M, Umapathi P, Arnson H, Rizo L, Mendell JR, Gage FH, Cleveland DW, Kaspar BK. Gene transfer demonstrates that muscle is not a primary target for non-cell-autonomous toxicity in familial amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A. 2006 Dec 19; 103(51):19546-51. PMID: 17164329; PMCID: PMC1748262.
    148. Weaver BA, Cleveland DW. Does aneuploidy cause cancer? Curr Opin Cell Biol. 2006 Dec; 18(6):658-67. PMID: 17046232.
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    149. Boillée S, Vande Velde C, Cleveland DW. ALS: a disease of motor neurons and their nonneuronal neighbors. Neuron. 2006 Oct 05; 52(1):39-59. PMID: 17015226.
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    150. Ross CA, Cleveland DW. Intercellular miscommunication in polyglutamine pathogenesis. Nat Neurosci. 2006 Oct; 9(10):1205-6. PMID: 17001335.
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    151. Miller TM, Smith RA, Cleveland DW. Amyotrophic lateral sclerosis and gene therapy. Nat Clin Pract Neurol. 2006 Sep; 2(9):462-3. PMID: 16932606.
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    152. Weaver BA, Silk AD, Cleveland DW. Cell biology: nondisjunction, aneuploidy and tetraploidy. Nature. 2006 Aug 17; 442(7104):E9-10; discussion E10. PMID: 16915240.
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    153. Smith RA, Miller TM, Yamanaka K, Monia BP, Condon TP, Hung G, Lobsiger CS, Ward CM, McAlonis-Downes M, Wei H, Wancewicz EV, Bennett CF, Cleveland DW. Antisense oligonucleotide therapy for neurodegenerative disease. J Clin Invest. 2006 Aug; 116(8):2290-6. PMID: 16878173; PMCID: PMC1518790.
    154. Yamanaka K, Miller TM, McAlonis-Downes M, Chun SJ, Cleveland DW. Progressive spinal axonal degeneration and slowness in ALS2-deficient mice. Ann Neurol. 2006 Jul; 60(1):95-104. PMID: 16802286.
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    155. Dhib-Jalbut S, Arnold DL, Cleveland DW, Fisher M, Friedlander RM, Mouradian MM, Przedborski S, Trapp BD, Wyss-Coray T, Yong VW. Neurodegeneration and neuroprotection in multiple sclerosis and other neurodegenerative diseases. J Neuroimmunol. 2006 Jul; 176(1-2):198-215. PMID: 16983747.
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    156. Boillée S, Yamanaka K, Lobsiger CS, Copeland NG, Jenkins NA, Kassiotis G, Kollias G, Cleveland DW. Onset and progression in inherited ALS determined by motor neurons and microglia. Science. 2006 Jun 02; 312(5778):1389-92. PMID: 16741123.
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    157. Eymard-Pierre E, Yamanaka K, Haeussler M, Kress W, Gauthier-Barichard F, Combes P, Cleveland DW, Boespflug-Tanguy O. Novel missense mutation in ALS2 gene results in infantile ascending hereditary spastic paralysis. Ann Neurol. 2006 Jun; 59(6):976-80. PMID: 16718699.
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    158. Foltz DR, Jansen LE, Black BE, Bailey AO, Yates JR, Cleveland DW. The human CENP-A centromeric nucleosome-associated complex. Nat Cell Biol. 2006 May; 8(5):458-69. PMID: 16622419.
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    159. Wang Z, Shah JV, Berns MW, Cleveland DW. In vivo quantitative studies of dynamic intracellular processes using fluorescence correlation spectroscopy. Biophys J. 2006 Jul 01; 91(1):343-51. PMID: 16603490; PMCID: PMC1479085.
    160. Yamanaka K, Cleveland DW. Determinants of rapid disease progression in ALS. Neurology. 2005 Dec 27; 65(12):1859-60. PMID: 16380607.
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    161. Bomont P, Maddox P, Shah JV, Desai AB, Cleveland DW. Unstable microtubule capture at kinetochores depleted of the centromere-associated protein CENP-F. EMBO J. 2005 Nov 16; 24(22):3927-39. PMID: 16252009; PMCID: PMC1283947.
    162. Kops GJ, Weaver BA, Cleveland DW. On the road to cancer: aneuploidy and the mitotic checkpoint. Nat Rev Cancer. 2005 Oct; 5(10):773-85. PMID: 16195750.
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    163. Mao Y, Desai A, Cleveland DW. Microtubule capture by CENP-E silences BubR1-dependent mitotic checkpoint signaling. J Cell Biol. 2005 Sep 12; 170(6):873-80. PMID: 16144904; PMCID: PMC2171436.
    164. Garcia ML, Singleton AB, Hernandez D, Ward CM, Evey C, Sapp PA, Hardy J, Brown RH, Cleveland DW. Mutations in neurofilament genes are not a significant primary cause of non-SOD1-mediated amyotrophic lateral sclerosis. Neurobiol Dis. 2006 Jan; 21(1):102-9. PMID: 16084104.
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    165. Lobsiger CS, Garcia ML, Ward CM, Cleveland DW. Altered axonal architecture by removal of the heavily phosphorylated neurofilament tail domains strongly slows superoxide dismutase 1 mutant-mediated ALS. Proc Natl Acad Sci U S A. 2005 Jul 19; 102(29):10351-6. PMID: 16002469; PMCID: PMC1177385.
    166. Weaver BA, Cleveland DW. Decoding the links between mitosis, cancer, and chemotherapy: The mitotic checkpoint, adaptation, and cell death. Cancer Cell. 2005 Jul; 8(1):7-12. PMID: 16023594.
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    167. Liu J, Shinobu LA, Ward CM, Young D, Cleveland DW. Elevation of the Hsp70 chaperone does not effect toxicity in mouse models of familial amyotrophic lateral sclerosis. J Neurochem. 2005 May; 93(4):875-82. PMID: 15857390.
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    168. Miller TM, Kaspar BK, Kops GJ, Yamanaka K, Christian LJ, Gage FH, Cleveland DW. Virus-delivered small RNA silencing sustains strength in amyotrophic lateral sclerosis. Ann Neurol. 2005 May; 57(5):773-6. PMID: 15852369; PMCID: PMC1351126.
    169. Beghi E, Bendotti C, Mennini T, Miller T, Cleveland D. Merits of a New Drug Trial for ALS? Science. 2005 Apr 29; 308(5722):632b-633b. PMID: 15863397.
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    170. Kops GJ, Kim Y, Weaver BA, Mao Y, McLeod I, Yates JR, Tagaya M, Cleveland DW. ZW10 links mitotic checkpoint signaling to the structural kinetochore. J Cell Biol. 2005 Apr 11; 169(1):49-60. PMID: 15824131; PMCID: PMC1351127.
    171. Miller TM, Cleveland DW. Medicine. Treating neurodegenerative diseases with antibiotics. Science. 2005 Jan 21; 307(5708):361-2. PMID: 15661995.
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    172. Vande Velde C, Cleveland DW. VEGF: multitasking in ALS. Nat Neurosci. 2005 Jan; 8(1):5-7. PMID: 15622408.
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    173. Wang H, Hu X, Ding X, Dou Z, Yang Z, Shaw AW, Teng M, Cleveland DW, Goldberg ML, Niu L, Yao X. Human Zwint-1 specifies localization of Zeste White 10 to kinetochores and is essential for mitotic checkpoint signaling. J Biol Chem. 2004 Dec 24; 279(52):54590-8. PMID: 15485811.
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    174. Lawrence CJ, Dawe RK, Christie KR, Cleveland DW, Dawson SC, Endow SA, Goldstein LS, Goodson HV, Hirokawa N, Howard J, Malmberg RL, McIntosh JR, Miki H, Mitchison TJ, Okada Y, Reddy AS, Saxton WM, Schliwa M, Scholey JM, Vale RD, Walczak CE, Wordeman L. A standardized kinesin nomenclature. J Cell Biol. 2004 Oct 11; 167(1):19-22. PMID: 15479732; PMCID: PMC2041940.
    175. Black BE, Foltz DR, Chakravarthy S, Luger K, Woods VL, Cleveland DW. Structural determinants for generating centromeric chromatin. Nature. 2004 Jul 29; 430(6999):578-82. PMID: 15282608.
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    176. Liu J, Lillo C, Jonsson PA, Vande Velde C, Ward CM, Miller TM, Subramaniam JR, Rothstein JD, Marklund S, Andersen PM, Brännström T, Gredal O, Wong PC, Williams DS, Cleveland DW. Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria. Neuron. 2004 Jul 08; 43(1):5-17. PMID: 15233913.
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    177. Shah JV, Botvinick E, Bonday Z, Furnari F, Berns M, Cleveland DW. Dynamics of centromere and kinetochore proteins; implications for checkpoint signaling and silencing. Curr Biol. 2004 Jun 08; 14(11):942-52. PMID: 15182667.
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    178. Kops GJ, Foltz DR, Cleveland DW. Lethality to human cancer cells through massive chromosome loss by inhibition of the mitotic checkpoint. Proc Natl Acad Sci U S A. 2004 Jun 08; 101(23):8699-704. PMID: 15159543; PMCID: PMC423258.
    179. Boillée S, Cleveland DW. Gene therapy for ALS delivers. Trends Neurosci. 2004 May; 27(5):235-8. PMID: 15111001.
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    180. Bruijn LI, Miller TM, Cleveland DW. Unraveling the mechanisms involved in motor neuron degeneration in ALS. Annu Rev Neurosci. 2004; 27:723-49. PMID: 15217349.
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    181. Vande Velde C, Garcia ML, Yin X, Trapp BD, Cleveland DW. The neuroprotective factor Wlds does not attenuate mutant SOD1-mediated motor neuron disease. Neuromolecular Med. 2004; 5(3):193-203. PMID: 15626820.
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    182. Yamanaka K, Vande Velde C, Eymard-Pierre E, Bertini E, Boespflug-Tanguy O, Cleveland DW. Unstable mutants in the peripheral endosomal membrane component ALS2 cause early-onset motor neuron disease. Proc Natl Acad Sci U S A. 2003 Dec 23; 100(26):16041-6. PMID: 14668431; PMCID: PMC307689.
    183. Garcia ML, Lobsiger CS, Shah SB, Deerinck TJ, Crum J, Young D, Ward CM, Crawford TO, Gotow T, Uchiyama Y, Ellisman MH, Calcutt NA, Cleveland DW. NF-M is an essential target for the myelin-directed "outside-in" signaling cascade that mediates radial axonal growth. J Cell Biol. 2003 Dec 08; 163(5):1011-20. PMID: 14662745; PMCID: PMC2173620.
    184. Miller TM, Cleveland DW. Has gene therapy for ALS arrived? Nat Med. 2003 Oct; 9(10):1256-7. PMID: 14520369.
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    185. Weaver BA, Bonday ZQ, Putkey FR, Kops GJ, Silk AD, Cleveland DW. Centromere-associated protein-E is essential for the mammalian mitotic checkpoint to prevent aneuploidy due to single chromosome loss. J Cell Biol. 2003 Aug 18; 162(4):551-63. PMID: 12925705; PMCID: PMC2173788.
    186. Mao Y, Abrieu A, Cleveland DW. Activating and silencing the mitotic checkpoint through CENP-E-dependent activation/inactivation of BubR1. Cell. 2003 Jul 11; 114(1):87-98. PMID: 12859900.
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    187. Xia CH, Roberts EA, Her LS, Liu X, Williams DS, Cleveland DW, Goldstein LS. Abnormal neurofilament transport caused by targeted disruption of neuronal kinesin heavy chain KIF5A. J Cell Biol. 2003 Apr 14; 161(1):55-66. PMID: 12682084; PMCID: PMC2172877.
    188. Cleveland DW, Mao Y, Sullivan KF. Centromeres and kinetochores: from epigenetics to mitotic checkpoint signaling. Cell. 2003 Feb 21; 112(4):407-21. PMID: 12600307.
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    189. Raoul C, Estévez AG, Nishimune H, Cleveland DW, deLapeyrière O, Henderson CE, Haase G, Pettmann B. Motoneuron death triggered by a specific pathway downstream of Fas. potentiation by ALS-linked SOD1 mutations. Neuron. 2002 Sep 12; 35(6):1067-83. PMID: 12354397.
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    190. Putkey FR, Cramer T, Morphew MK, Silk AD, Johnson RS, McIntosh JR, Cleveland DW. Unstable kinetochore-microtubule capture and chromosomal instability following deletion of CENP-E. Dev Cell. 2002 Sep; 3(3):351-65. PMID: 12361599.
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    191. Rao MV, Garcia ML, Miyazaki Y, Gotow T, Yuan A, Mattina S, Ward CM, Calcutt NA, Uchiyama Y, Nixon RA, Cleveland DW. Gene replacement in mice reveals that the heavily phosphorylated tail of neurofilament heavy subunit does not affect axonal caliber or the transit of cargoes in slow axonal transport. J Cell Biol. 2002 Aug 19; 158(4):681-93. PMID: 12186852.
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    192. Subramaniam JR, Lyons WE, Liu J, Bartnikas TB, Rothstein J, Price DL, Cleveland DW, Gitlin JD, Wong PC. Mutant SOD1 causes motor neuron disease independent of copper chaperone-mediated copper loading. Nat Neurosci. 2002 Apr; 5(4):301-7. PMID: 11889469.
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    193. Shah JV, Cleveland DW. Slow axonal transport: fast motors in the slow lane. Curr Opin Cell Biol. 2002 Feb; 14(1):58-62. PMID: 11792545.
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    194. Howland DS, Liu J, She Y, Goad B, Maragakis NJ, Kim B, Erickson J, Kulik J, DeVito L, Psaltis G, DeGennaro LJ, Cleveland DW, Rothstein JD. Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS). Proc Natl Acad Sci U S A. 2002 Feb 05; 99(3):1604-9. PMID: 11818550; PMCID: PMC122237.
    195. Wataya T, Nunomura A, Smith MA, Siedlak SL, Harris PL, Shimohama S, Szweda LI, Kaminski MA, Avila J, Price DL, Cleveland DW, Sayre LM, Perry G. High molecular weight neurofilament proteins are physiological substrates of adduction by the lipid peroxidation product hydroxynonenal. J Biol Chem. 2002 Feb 15; 277(7):4644-8. PMID: 11733539.
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    196. Cleveland DW, Rothstein JD. From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS. Nat Rev Neurosci. 2001 Nov; 2(11):806-19. PMID: 11715057.
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    197. Cleveland DW. From Charcot to SOD1: mechanisms of selective motor neuron death in ALS. Neuron. 1999 Nov; 24(3):515-20. PMID: 10595505.
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    198. Corson LB, Strain JJ, Culotta VC, Cleveland DW. Chaperone-facilitated copper binding is a property common to several classes of familial amyotrophic lateral sclerosis-linked superoxide dismutase mutants. Proc Natl Acad Sci U S A. 1998 May 26; 95(11):6361-6. PMID: 9600970; PMCID: PMC27707.