Ellerby Lab

1. Fink AL, Ellerby LM, Bassett, PM. Conformational Changes in the Inactivation of ß-lactamase. J. Am. Chem. Soc. 111:6871-6873, 1989. Download PDF file 368.81 Kb

2. Ellerby LM, Escobar WA, Fink AL, Wells JA. The Role of Lysine-234 in ß-lactamase Catalysis Probed by Site-Directed Mutagenesis. Biochemistry 29:5797-5806, 1990. Download PDF file 1.18 Mb

3. Yamanaka SA, Nishida F, Nishida C, Ellerby LM, Dunn B, Valentine JS, Zink JI. Enzymatic Activity of Glucose Oxidase Encapsulated in Transparent Glass by the Sol-Gel Method. Chem. Materials 4:495-497, 1992. Download PDF file 389.85 Kb

4. Ellerby LM, Nishida CR, Nishida F, Yamanaka SA, Dunn B, Valentine JS, Zink JI. Encapsulation of Proteins in Transparent Porous Glasses Prepared by the Sol-Gel Method. Science 255:1113-1115, 1992

5. Wu S, Ellerby LM, Cohan JS, Dunn B, El-Sayed MA, Valentine JS, Zink JI. Bacteriorhodopsin Encapsulated in Transparent Sol-Gel Glass: A New Biomaterial. Chem. Materials 5:115-120, 1993. Download PDF file 742.45 Kb

6. Tamai KT, Gralla EB, Ellerby LM, Valentine JS, Thiele DJ. Yeast and Mammalian Metallothioneins Functionally Substitute for Yeast Copper-Zinc Superoxide Dismutase. Proc. Natl. Acad. Sci. U S A. 90:8013-8017, 1993. Download PDF file 1.46 Mb

7. Graden JA, Ellerby LM, Roe JA, Valentine JS. Role of the Bridging Histidyl Imidazolate Ligand in Yeast Copper-Zinc Superoxide Dismutase. Characterization of the His63Ala Mutant. J. Am. Chem. Soc. 116:9743-9744, 1994. Download PDF file 255.40 Kb

8. Ellerby LM, Ellerby HM, Park SM, Holleran AL, Murphy AN, Fiskum G, Kane DJ, Testa MP, Kayalar C, Bredesen DE. Shift of the Cellular Oxidation-reduction Potential in Neural Cells Expressing Bcl-2. J. Neurochem. 67:1259-1267, 1996. Download PDF file 1.16 Mb

9. Ellerby LM, Cabelli DE, Graden JA, Valentine JS. Copper-zinc Superoxide Dismutase: Why not pH-Dependent? J. Am. Chem. Soc. 118:6556-6561, 1996. Download PDF file 182.52 Kb

10. Schultz JR, Ellerby LM, Gralla EB, Valentine JS, Clarke, CF. Autoxidation of Ubiquinol-6 is Independent of Superoxide Dismutase. Biochemistry 35:6595-6603, 1996. Download PDF file 370.19 Kb

11. Lyons TJ, Hongbin L, Goto JJ, Nersissian A, Roe JA, Graden JA, Cafe, C, Ellerby LM, Bredesen DE, Gralla EB, Valentine JS. Mutations in Copper-zinc Superoxide Dismutase that Cause Amyotrophic Lateral Sclerosis Alter the Zinc Binding Site and Redox Behavior of the Protein. Proc. Natl. Acad. Sci. U S A. 93:12240-12244, 1996.

12. Bredesen DE, Wiedau-Pazos M, Goto JJ, Rabizadeh S, Roe JA, Gralla EB, Ellerby LM, Valentine JS. Cell Death Mechanisms in ALS. Neurology 47/4 Suppl. 2:S36-S38, 1996.

13. Longo V, Ellerby LM, Bredesen DE, Valentine JS, Gralla EB. Human Bcl-2 Reverses Survival Defects in Yeast Lacking Superoxide Dismutase and Delays Death of Wild type Yeast. J. Cell Biol. 137:1581-1588, 1997.

14. Ellerby HM, Martin SJ, Ellerby LM, Naiem SS, Rabizadeh S, Salvesen GS, Casciano C, Cashman NR, Green DR, Bredesen DE. Establishment of a Cell-free System for Neuronal Apoptosis: Comparison of Pre-mitochondrial, Mitochondrial, and Post-mitochondrial Phases. J. Neurosci. 17:6165-6178, 1997.

15. Bredesen DE, Ellerby LM, Hart PJ, Wiedau-Pazos M, Valentine JS. Do Post-translational Modifications of CuZnSOD Lead to Sporadic ALS? Ann. Neurol. 42:135-137, 1997.

16. Martindale D, Hackam A, Wieczorek A, Ellerby LM, Wellington C, McCutcheon K, Singaraja R, Kazemi-Esfarjani P, Devon R, Kim SU, Bredesen D, Tufaro F, Hayden MR. Length of Huntingtin and its Polyglutamine Tract Influences Localization and Frequency of Intracellular Aggregates. Nature Genet. 18:150-154, 1998.

17. Jurgensmeier JM, Xie Z, Deveraux Q, Ellerby LM, Bredesen D, Reed JC. Bax Directly Induces Release of Cytochrome c from Isolated Mitochondria. Proc. Natl. Acad. Sci. U S A. 95:4997-5002, 1998. Download PDF file 277.93 Kb

18. Wellington CL, Ellerby LM, Hackam AS, Margolis RL, Trifiro MA, Singaraja R, McCutcheon K, Salvesen GS, Propp SS, Bromm M, Rowland KJ, Zhang T, Rasper D, Roy S, Thornberry N, Pinsky L, Kakizuka A, Ross CA, Nicholson DW, Bredesen DE, Hayden MR. Caspase Cleavage of Gene Products Associated with Triplet Expansion Disorders Generates Truncated Fragments Containing the Polyglutamine Tract. J. Biol. Chem. 273:9158-9167, 1998.

19. Stennicke HR, Jurgensmeier JM, Shin, H, Deveraux Q, Wolf BB, Yang X, Zhou Q, Ellerby HM, Ellerby LM, Bredesen D, Green DR, Reed JC, Froelich CJ, Salvesen GS. Pro-caspase-3 is a Major Physiologic Target of Caspase-8. J. Biol. Chem. 273:27084-27090, 1998.

20. Ellerby LM, Hackam AS, Propp SS, Ellerby HM, Rabizadeh S, Cashman NR, Trifiro MA, Pinsky L, Wellington CL, Salvesen GS, Hayden MR, Bredesen DE. Kennedy's Disease: Caspase Cleavage of the Androgen Receptor Is a Crucial Event in Cytotoxicity. J. Neurochem. 72:185-195, 1999. Download PDF file 294.87 Kb

21. Ellerby LM, Andrusiak RL, Wellington CL, Hackam AS, Propp SS, Wood JD, Sharp AH, Margolis RL, Ross CA, Salvesen GS, Hayden MR, Bredesen DE. Cleavage of Atrophin-1 at Caspase Site Aspartic Acid 109 Modulates Cytotoxicity. J. Biol. Chem. 274:8730-8736, 1999.

22. Krajewski S, Krajewska M, Ellerby LM, Welsh K, Xie Z, Deveraux QL, Salvesen GS, Bredesen DE, Rosenthal RE, Fiskum G, Reed JC. Release of Caspase-9 from Mitochondria During Neuronal Apoptosis and Cerebral Ischemia. Proc. Natl. Acad. Sci. U S A. 96:5752-5757, 1999.

23. Ellerby HM, Arap W, Ellerby LM, Kain R, Andrusiak R, Rio GD, Krajewski S, Lombardo CR, Rao R, Ruoslahti E, Bredesen DE, Pasqualini R. Anti-Cancer Activity of Targeted Pro-Apoptotic Peptides. Nat. Med. 5:1032-1038, 1999. Download PDF file 765.44 Kb

24. Cafe C, Testa MP, Sheldon PJ, French WP, Ellerby LM, Bredesen DE. Loss of Oxidation-Reduction Specificity in Amyotrophic Lateral Sclerosis-Associated CuZnSOD Mutants. J. Mol. Neurosci. 15:71-83, 2000. Download PDF file 993.30 Kb

25. Rabizadeh S, Ye X, Sperandio S, Wang JJ, Ellerby HM, Ellerby LM, Giza C, Andrusiak RL, Frankowski H, Yaron Y, Moayeri NN, Rovelli G, Evans CJ, Butcher LL, Nolan GP, Assa-Munt N, Bredesen DE. Neurotrophin Dependence Domain: A Domain Required for the Mediation of Apoptosis by the p75 Neurotrophin Receptor. J. Mol. Neurosci. 15:215-229, 2000. Download PDF file 282.69 Kb

26. Ellerby LM, Bredesen DE. Measurement of Cellular Oxidation, Reactive Oxygen Species, and Antioxidant Enzymes During Apoptosis. Methods Enzymol. 322:413-421, 2000. Download PDF file 1.00 Mb

27. Lu DC, Rabizadeh S, Chandra S, Shayya RF, Ellerby LM, Ye X, Salvesen GS, Koo EH, Bredesen DE. A Second Cytotoxic Proteolytic Peptide Derived from Amyloid b- Protein Precursor. Nat. Med. 6:397-404, 2000. Download PDF file 434.63 Kb

28. Wellington CL, Singaraja R, Ellerby L, Savill J, Roy S, Leavitt B, Cattaneo E, Hackam A, Sharp A, Thornberry N, Nicholson DW, Bredesen DE, Hayden MR. Inhibiting Caspase Cleavage of Huntingtin Reduces Toxicity and Aggregate Formation in Neuronal and Nonneuronal Cells. J. Biol. Chem. 275:19831-19838, 2000. Download PDF file 373.29 Kb

29. Kluck RM, Ellerby LM, Ellerby HM, Naiem S, Yaffe MP, Margoliash E, Bredesen D, Mauk AG, Sherman F, Newmeyer DD. Determinants of Cytochrome C Pro-Apoptotic Activity. The Role of Lysine 72 Trimethylation. J. Biol. Chem. 275:16127-16133, 2000.

30. Stoka V, Turk B, Schendel SL, Kim TH, Cirman T, Snipas SJ, Ellerby LM, Bredesen D, Freeze H, Abrahamson M, Bromme D, Krajewski S, Reed JC, Yin XM, Turk V, Salvesen GS. Lysosomal Protease Pathways to Apoptosis. Cleavage of Bid, Not Pro-Caspases, is the Most Likely Route. J. Biol. Chem. 276:3149-3157, 2001.

31. Peel AL, Rao RV, Cottrell BA, Hayden MR, Ellerby LM, Bredesen DE. Double-Stranded RNA-Dependent Protein Kinase, PKR, Binds Preferentially to Huntington's Disease (HD) Transcripts and is Activated in HD Tissue. Hum. Mol. Genet. 10:1531-1538, 2001.

32. Rao RV, Hermel E, Castro-Obregon S, del Rio G, Ellerby LM, Ellerby HM, Bredesen DE. Coupling Endoplasmic Reticulum Stress to the Cell Death Program. Mechanism of Caspase Activation. J. Biol. Chem. 276:33869-33874, 2001.

33. Lee M, Hyun DH, Marshall KA, Ellerby LM, Bredesen DE, Jenner P, Halliwell B. Effect of Overexpression of Bcl-2 on Cellular Oxidative Damage, Nitric Oxide Production, Antioxidant Defenses, and the Proteasome. Free Radic. Biol. Med. 31:1550-1559, 2001. Download PDF file 242.59 Kb

34. Rao RV, Peel A, Logvinova A, del Rio G, Hermel E, Yokota T, Goldsmith PC, Ellerby LM, Ellerby HM, Bredesen DE. Coupling Endoplasmic Reticulum Stress to the Cell Death Program: Role of the ER Chaperone GRP78. FEBS Lett. 514:122-128, 2002. Download PDF file 523.41 Kb

35. Gafni J, Ellerby LM. Calpain Activation in Huntington's Disease. J. Neurosci. 22:4842-4849, 2002.

36. Wellington CL*, Ellerby LM*, Gutekunst CA, Rogers D, Warby S, Graham RK, Loubser O, van Raamsdonk J, Singaraja R, Yang YZ, Gafni J, Bredesen D, Hersch SM, Leavitt BR, Roy S, Nicholson DW, Hayden MR. Caspase Cleavage of Mutant Huntingtin Precedes Neurodegeneration in Huntington's Disease. J. Neurosci. 22:7862-7872, 2002. *Co-first Authors.

37. Ellerby LM. Hunting for Excitement: NMDA Receptors in Huntington's Disease. Neuron 33:841-842, 2002. Download PDF file 33.38 Kb

38. Nucifora FC, Ellerby LM, Wellington CL, Wood JD, Herring WJ, Sawa A, Hayden MR, Dawson VL, Dawson TM, Ross CA. Nuclear Localization of a Non-Caspase Truncation Product of Atrophin-1, with an Expanded Polyglutamine Repeat, Increases Cellular Toxicity. J. Biol. Chem. 278:13047-13055, 2003.

39. Kaur D, Yantiri F, Rajagopalan S, Kumar J, Mo JQ, Boonplueang R, Viswanath V, Jacobs R, Yang L, Beal MF, DiMonte D, Volitaskis I, Ellerby L, Cherny RA, Bush AI, Andersen JK. Genetic or Pharmacological Iron Chelation Prevents MPTP-Induced Neurotoxicity in Vivo: A Novel Therapy for Parkinson's Disease. Neuron 37:899-909, 2003. Download PDF file 289.95 Kb

40. Ellerby HM, Lee S, Ellerby LM, Chen S, Kiyota T, Del Rio G, Sugihara G, Sun Y, Bredesen DE, Arap W and Pasqualini R. An Artificially Designed Pore-Forming Protein with Anti-Tumor Effects. J. Biol. Chem. 278:35311-35316, 2003.

41. LaFevre-Bernt MA, Ellerby LM. Kennedy's Disease. Phosphorylation of the Polyglutamine-Expanded Form of Androgen Receptor Regulates Its Cleavage by Caspase-3 and Enhances Cell Death. J. Biol. Chem. 278:34918-34924 2003.

42. Wellington CL, Ellerby LM, Leavitt BR and Hayden MR. Huntington Proteolysis in Huntington Disease. Clinical Neuroscience Research 3:129-139, 2003. Download PDF file 315.58 Kb

43. Sopher BL, Thomas PS, Jr., LaFevre-Bernt MA, Holm IE, Wilke SA, Ware CB, Jin LW, Libby RT, Ellerby LM, La Spada AR. Androgen Receptor YAC Transgenic Mice Recapitulate SBMA Motor Neuronopathy and Implicate VEGF164 in the Motor Neuron Degeneration. Neuron 41:687-99, 2004. Download PDF file 568.79 Kb

44. Shoesmith Berke SJ, Flores Schmied FA, Brunt ER, Ellerby LM, Paulson HL. Caspase-mediated Proteolysis of the Polyglutamine Disease Protein Ataxin-3. J. Neurochem. 89:908-918, 2004. Download PDF file 531.86 Kb

45. Gafni, J., Hermel, E. Young, J.E., Wellington, C.L., Hayden, M.R., and Ellerby L.M. Inhibition of Calpain Cleavage of Huntingtin Reduces Toxicity: Accumulation of Calpain/Caspase Fragments in the Nucleus, J. Biol. Chem. 279:20211-20220, 2004.

46. Hermel E, Gafni J, Propp S, Leavitt B, Wellington CL, Hackam A, Logvinova A, Peel A, Chen S, Singaraja R, Karjewski S, Goldsmith PC, Ellerby HM, Salvesen GS, Hayden MR, Bredesen DE, Ellerby LM. Specific Caspase Interactions and Amplification Are Involved in Selective Neuronal Vulnerability in Huntington's Disease. Cell Death Differ. 11:424-438, 2004.

47. Jin K, Lafevre-Bernt M, Sun Y, Chen S, Gafni J, Crippen D, Logvinova A, Ross CA, Greenberg DA, Ellerby LM. Fgf-2 Promotes Neurogenesis and Neuroprotection and Prolongs Survival in a Transgenic Mouse Model of Huntington's Disease. Proc. Natl. Acad. Sci. U S A. 102:18189-94, 2005.

48. Iwata A, Christianson JC, Bucci M, Ellerby LM, Nukina N, Forno LS, Kopito RR. Increased Susceptibility of Cytoplasmic over Nuclear Polyglutamine Aggregates to Autophagic Degradation. Proc. Natl. Acad. Sci. U S A. 102:13135-40, 2005.

49. Tanaka Y, Igarashi S, Nakamura M, Gafni J, Torcassi C, Schilling G, Crippen D, Wood JD, Sawa A, Jenkins NA, Copeland NG, Borchelt DR, Ross CA, Ellerby LM. Progressive Phenotype and Nuclear Accumulation of an Amino-Terminal Cleavage Fragment in a Transgenic Mouse Model with Inducible Expression of Full-Length Mutant Huntingtin. Neurobiol. Dis. 21:381-91, 2006. Download PDF file 463.73 Kb

50. Taylor J, Grote SK, Xia J, Vandelft M, Graczyk J, Ellerby LM, La Spada AR, Truant R. Ataxin-7 Can Export from the Nucleus Via a Conserved Exportin-Dependent Signal. J. Biol. Chem. 281:2730-9, 2006.

51. Sandoval CM, Geierstanger BH, Fujimura S, Balatbat C, Williams T, de Unamuno J, Whiles-Lillig JA, Ellerby LM, Michael Ellerby H, Jennings P, Plesniak LA. Structural Evaluation of a Novel Pro-Apoptotic Peptide Coupled to CNGRC Tumor Homing Sequence by NMR. Chem. Biol. Drug Des. 67:417-24, 2006. Download PDF file 332.84 Kb

52. Schilling B, Gafni J, Torcassi C, Row RH, LaFevre-Bernt MA, Cusak MP, Ratovitski T, Hirschhorn R, Ross CA, Gibson BW, Ellerby LM. Huntingtin Phosphorylation Sites Mapped by Mass Spectrometry: Modulation of Cleavage and Toxicity. J. Biol. Chem. 281:23686-97, 2006.

53. Ellerby LM, Orr HT, Neurodegenerative Disease: Cut to the Chase. Nature 442:641-642, 2006. Download PDF file 148.55 Kb

54. Oliveira JMA, Chen S, Almeida S, Riley R, Gonclaves J, Oliveira CR, Hayden MR, Nicholls DG, Ellerby LM, Rego AC. Mitochondrial-dependent Ca2+-handling in Huntington's Disease Striatal Cells: Effect of HDAC Inhibitors. J. Neurosci. 26:11174-86, 2006.

55. Oliveira JMA, Jekabsons M, Chen S, Lin A, Rego C, Ellerby LM, Nicholls D. Mitochondrial Dysfunction in Huntington's Disease: The Bioenergetics of Isolated and in-Situ Mitochondria from Transgenic Mice. J. Neurochem. 101:241-249, 2007. Download PDF file 580.30 Kb

56. Khan A, Sun Y, Jin K, Mao XO, Chen S, Ellerby LM, Greenberg DA: A neuroglobin-overexpressing transgenic mouse. GENE, in press. Download PDF file 1.95 Mb

57. Khan AA, Wang Y, Sun Y, Mao XO, Xie L, Miles E, Graboski J, Chen S, Ellerby LM, Jin K, Greenberg DA. Neuroglobin-overexpressing transgenic mice are resistant to cerebral and myocardial ischemia. Proc. Natl. Acad. Sci. U S A. 103:17944-8, 2006.

58. Bennett EJ, Shaler T, Gonzalez-Zulueta M, Schulman HF, Iwata A, Riley BE, Johnston JA, Bucci M, Nukina N, Ellerby L, Kopito RR. Cellular mechanisms of protein quality control. Rinsho Shinkeigaku. 46:805, 2006.

59. Kaltenbach LS, Romero E, Becklin RR, Chettier R, Bell R, Phansalkar A, Strand A, Torcassi C, Savage J, Hurlburt A, Cha GH, Ukani L, Chepanoske CL, Zhen Y, Sahasrabudhe S, Olson J, Kurschner C, Ellerby LM, Peltier JM, Botas J, Hughes RE. Huntingtin Interacting Proteins Are Genetic Modifiers of Neurodegeneration. PLoS Genet. 11;3(5):e82, 2007.

60. Young JE, Gouw L, Propp S, Sopher BL, Taylor J, Lin A, Hermel E, Logvinova A, Chen SF, Chen S, Bredesen DE, Truant R, Ptacek LJ, La Spada AR, Ellerby LM. Proteolytic Cleavage of Ataxin-7 by Caspase-7 Modulates Cellular Toxicity and Cleavage Correlates with in Vivo Ataxin-7 Proteolysis. J. Biol. Chem. 282:30150-60, 2007.

61. Young JE, Garden GA, Martinez RA, Tanaka F, Sandoval CM, Smith AC, Sopher BL, Lin A, Fischbeck KH, Ellerby LM, Morrison RS, Taylor JP, La Spada AR. Polyglutamine-expanded androgen receptor truncation fragments activate a Bad-dependent apoptotic cascade mediated by DP5/Hrk. J. Neurosci. 29:1987-97, 2009. PMCID: PMC2746676.

62. Gafni J, Cong X, Chen SF, Gibson BW, Ellerby LM. Calpain-1 cleaves and activates caspase-7. J. Biol. Chem. 284:25441-9, 2009. PMC Journal - In Process.

63. Papanikolaou T, Ellerby LM. IGF-1: elixir for motor neuron diseases. Neuron 63:277-8, 2009. PMC Journal - In Process.

64. Mookerjee S, Papanikolaou T, Guyenet SJ, Sampath V, Lin A, Vitelli C, DeGiacomo F, Sopher BL, Chen SF, La Spada AR, Ellerby LM. Posttranslational Modification of Ataxin-7 at Lysine 257 Prevents Autophagy-Mediated Turnover of an N-Terminal Caspase-7 Cleavage Fragment. J. Neurosci. 29:15134-15144, 2009. PMC Journal - In Process.

65. Duncan C, Papanikolaou T, Ellerby LM. Autophagy: PolyQ toxic fragment turnover. Autophagy, 6:Epub, 2010. PMC Journal - In Process.

66. Landles C, Sathasivam K, Weiss A, Woodman B, Moffitt H, Finkbeiner S, Sun B, Gafni J, Ellerby LM, Trottier Y, Richards WG, Osmand A, Paganetti P, Bates GP. Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntingtons disease. J. Biol. Chem., Epub, 2010. PMC Journal - In Process.