Orna Elroy-Stein, Ph.D. Tel Aviv University PUBLICATIONS 1. Levanon, D., J. Leiman-Hurwitz, N. Dafni, M. Wigderson, Z. Laver-Rudich, E. Danciger, O. Elroy-Stein, Y. Groner. (1985) Architecture and anatomy of the chromosomal locus in human chromosome 21 encoding the Cu/Zn-superoxide dismutase. EMBO J. 4, 77-84. 2. Groner, Y., O. Elroy-Stein, Y. Bernstein, N. Dafni, D. Levanon, E. Danciger, A. Neer. Molecular genetics of Down's syndrome: Overexpression of transfected human Cu/Znsuperoxide dismutase gene and the consequent physiological changes. New York, Cold Spring Harbor Sym. Quant. Biol. Vol.51, 1986 (pp.381-393). 3. Elroy-Stein, O., Y. Bernstein, Y. Groner. (1987) Overproduction of human Cu/Znsuperoxide dismutase in transfected cells: Extenuation of paraquate-mediated cytotoxicity and enhancement of lipid peroxidation. EMBO J. 5, 616-622. 4. Epstein, C.J., K.B. Avraham, M. Lovett, S. Smith, O. Elroy-Stein, G. Rotman, C. Bry, Y. Groner. (1987) Transgenic mice with increased Cu/Zn-superoxide dismutase activity: Animal model of dosage effect in Down's syndrome. Proc. Natl. Acad. Sci. USA 84, 80448048. 5. Elroy-Stein, O. and Y. Groner. (1988) Impaired neurotransmitter uptake in rat PC12 cells overexpressing human Cu/Zn-superoxide dismutase - Implication for dosage effect in Down's syndrome. Cell 52, 259-267. 6. Schickler, M., H. Knobler, K.B. Avraham, O. Elroy-Stein, Y. Groner, (1989) Diminished serotonin uptake in platelets of transgenic mice with increased Cu/Zn-superoxide dismutase activity. EMBO J. 8, 1385-1392. 7. Elroy-Stein, O., T.R. Fuerst, B. Moss. (1989) Cap-independent translation of mRNA conferred by encephalomyocarditis virus 5' sequence improves the performance of the vaccinia virus/bacteriophage T7 hybrid expression system. Proc. Natl. Acad. Sci. USA 86, 6126-6130. 8. Groner, Y., O. Elroy-Stein, K.B. Avraham, R. Yarom, M. Schickler, H. Knobler, G. Rotman. (1990) Down syndrome clinical symptoms are manifested in transfected cells and transgenic mice overexpressing the human Cu/Zn-superoxide dismutase gene. J. Physiol. 84, 53-77. 9. Elroy-Stein, O. and B. Moss. (1990) Cytoplasmic expression system based on constitutive synthesis of bacteriophage T7 RNA polymerase in mammalian cells. Proc. Natl. Acad. Sci. USA 87, 6743-6747. 10. Moss, B., O. Elroy-Stein, T. Mizukami, W.A. Alexander, T.R. Fuerst. (1990) New mammalian expression vectors. Nature 348, 91-92. 11. Zibert, A., H-C. Selinka, O. Elroy-Stein, B. Moss, E. Wimmer. (1991) Vaccinia Virus mediated Expression and Identification of the Human Poliovirus Receptor. Virology 182, 250-259. 12. Yang, X-C., A. Karschin, C. Labarca, O. Elroy-Stein, B. Moss, N. Davidson, H.N. Lester. (1991) Expression of ion channels and receptors in Xenopus oocytes using vaccinia virus. FASEB J. 5, 2209-2216. Orna Elroy-Stein, Ph.D. Tel Aviv University 13. Karschin, A., B.Y. Ho, C. Labarca, O. Elroy-Stein, B. Moss, N. Davidson, H.A. Lester. (1991) Heterologously expressed serotonin 1A receptors couple to muscarinic K+ channel in heart. Proc. Natl. Acad. Sci. USA. 88, 5694-5698. 14. Yang, X.-C., C. Labarca, J. Nargeot, B.Y. Ho, O. Elroy-Stein, B. Moss, N. Davidson, H.A. Lester. (1992) Cell specific posttranslational events affect functional expression at the plasma membrane but not tetrodotoxin sensitivity of rat brain IIA sodium channel alpha subunit expressed in mammalian cells. J. Neuroscience 12, 268-277. 15. Morgan,R.A., L. Couture, O. Elroy-Stein, J. Ragheb, B. Moss, W.F. Anderson. (1992) Retroviral vectors containing putative internal ribosome entry sites: development of a polycistronic gene transfer system and application to human gene therapy. Nucleic Acids Research 20, 1293-1299. 16. Davies, M.V., O. Elroy-Stein, R, Jagus, B. Moss, R.J. Kaufman. (1992) The vaccinia K3L gene product potentiates translation by inhibiting double- stranded-RNA-activated protein kinase and phosphorylation of the alpha subunit of eukaryotic initiation factor 2. J. Virol. 66, 1943-1950. 17. Zibert, A., H.C. Selinka, O. Elroy-Stein, E. Wimmer. (1992) The soluble form of two Nterminal domains of the poliovirus receptor is sufficient for blocking viral infection. Virus Research 25, 51-61. 18. Carroll, K., O. Elroy-Stein, B. Moss, R. Jagus. (1993) Recombinant vaccinia virus K3L gene product prevents activation of dsRNA-dependent, eIF-2a specific protein kinase. J. Biol. Chem. 268, 12837-12842. 19. Whetter, L.E., S.P. Day, O. Elroy-Stein, E.A. Brown, S.M. Lemon. (1994) Low efficiency of the 5' Nontranslated Region of Hepatitis A RNA is Promoting Cap-Independent Translation in Permissive Monkey Kidney Cells. J. Virol. 68, 5253-5263. 20. Whetter, L.E., S.P. Day, E.A. Brown, O. Elroy-Stein, S.M. Lemon. (1994) Analysis of hepatitis A virus translation in a T7 polymerase-expressing cell line. Arch. Virol. 9, 291298. 21. Zrihan-Licht, S., H.L. Vos, A. Baruch, O. Elroy-Stein, D. Sagiv, I. Keydar, J. Hilkens, D.H. Wreschner. (1994) Characterization and molecular cloning of a novel MUC1 protein devoid of tandem repeats expressed in human breast cancer tissue. Eur. J. Biochem. 224, 787-795. 22. Zrihan-Licht, S., A. Baruch, O. Elroy-Stein, I. Keydar, D.H. Wreschner. (1994) Tyrosine phosphorylation of the MUC1 breast cancer membrane proteins: Cytokine receptor-like molecules. FEBS Lett. 356, 130-136. 23. Bernstein, J., I. Sheffler, O. Elroy-Stein. (1995) The translational repression mediated by the PDGF/c-sis mRNA leader is relieved during megakaryocytic differentiation. J. Biol. Chem. 270, 10559-10565. 24. Shterman, N., O. Elroy-Stein, I. Morad, M. Amitsur, G. Kaufmann. Cleavage of the HIV replication primer tRNA lys,3 in human cells expressing bacterial anticodon nuclease. (1995) Nucleic Acids Res. 23, 1744-1749. 25. Pasmanik-Chor, M., O. Elroy-Stein, S. Gatt, H. Aerts, M. Horowitz. (1996) Overexpression of human glucocerebrosidase containing different size leaders. Biochem. J. Vol. 316, 81-88. 2 Orna Elroy-Stein, Ph.D. Tel Aviv University 26. Pasmanik-Chor, M., S. Laadan, O. Elroy-Stein, A. Zimran, A. Abrahamov, S. Gatt, M. Horowitz. (1996) The glucocerebrosidase D409H mutation in Gaucher disease. Biochem. & Molec. Med. 59, 125-133. 27. Strauss, D., O. Elroy-Stein, R. Ehrlich. (1997) Adenovirus E1A interferes with expression of vaccinia viral genes. Gene. 184, 279-284. 28. Bernstein, J., O. Sella, S-Y. Le, O. Elroy-Stein. (1997) PDFG2/c-sis mRNA leader contains a differentiation-induced internal ribosomal entry site (D-IRES). J. Biol. Chem. 272, 93569362. 29. Savitsky, K., M. Platzer, T. Usiel, S. Gilad, A. Sartiel, A. Rosenthal, O. Elroy-Stein, Y. Shiloh, G. Rotman. (1997) Ataxia-telangiectasia: Structural diversity of untranslated sequences suggests complex post-transcriptional regulation of ATM gene expression. Nucleic Acid Res. 25, 1678-1684. 30. Pasmanik-Chor, M., L. Madar-Shapiro, O. Elroy-Stein, H. Aerts, S. Gatt, M. Horowitz. (1997) Expression of human mutated glucocerebrosidase alleles in human cells. Hum. Mol. Genet. 6, 887-895. 31. Gerlitz, G. and O. Elroy-Stein. (1997) Does EBV modulates ADA mRNA translation? Leukemia 11, 249. 32. Akiri, G., D. Nahari, Y. Finkelstein, S.-Y. Le, O. Elroy-Stein, B.-Z. Levi. (1998) Regulation of vascular endothelial growth factor (VEGF) expression is mediated by internal initiation of translation and alternative initiation of transcription. Oncogene 17, 227-237. 33. Sella, O., S-Y. Le, G. Gerlitz, O. Elroy-Stein. (1999) Differentiation-induced internal translation of PDGF2/c-cis mRNA: analysis of the cis-elements and their differentiationlinked binding to hnRNP-C. Molec. Cell Biol. 19, 5429-5440. 34. Finkelstein, Y., O. Factor, O. Elroy-Stein, B.Z. Levi. (1999) The use of bi-cistronic transfer vectors for the baculovirus expression sytem. J. Biotechnol. 75, 33-44 35. Pozner, A., V. Negreanu, D. Goldenberg, S.-Y. Le, O. Elroy-Stein, D. Levanon, Y. Groner. (2000) Transcription Coupled-Translation Control of AML1/RUNX1 is Mediated by Capand IRES-dependent Mechanisms. Molec. Cell. Biol. 20, 2297-2307 36. Gerlitz, G., R. Jagus, O. Elroy-Stein. (2002) Phosphorylation of initiation factor 2 is required for activation of internal translation initiation during cell differentiation. Eur. J. Biochem. 269, 2810-2819. 37. Koloteva-Levine, N., M. Amichay, O. Elroy-Stein. (2002) Interaction of hnRNP-C1/C2 proteins with RNA: analysis using the yeast three-hybrid system. FEBS Letters 523, 73-78; 38. Koloteva-Levine, N., D. Pinchasi, I. Pereman, A. Zur, M. Brandeis, O. Elroy-Stein. (2004) The Apc5 subunit of APC/Cyclosome interacts with PABP and represses IRES-mediated translation. Molec. Cell. Biol. 24, 3577-3587 3 Orna Elroy-Stein, Ph.D. Tel Aviv University 39. Dahary, D., O. Elroy-Stein, R. Sorek (2005) Naturally occurring antisense: Transcriptional leakage or real overlap? Genome Research 15, 364-368 40. Kantor, L., H. P. Harding, D. Ron, R. Schiffmann, C. R. Kaneski, S. Kimball, O. ElroyStein. (2005) Heightened stress response in primary fibroblasts expressing mutant eIF2B genes from CACH/VWM leukodystrophy patients. Hum. Genet. 118, 99-106 41. Schiffmann, R. and O. Elroy-Stein. (2006) Childhood Ataxia with CNS Hypomyelination/Vanishing White Matter Disease - A Common Leukodystrophy Caused by Abnormal Control of Protein Synthesis. Mol Genet Metab. 88, 7-15 42. Silvera, D., N. Koloteva-Levine, S. Burma, O. Elroy-Stein. (2006) Effect of Ku proteins on IRES-mediated translation. Biol Cell 98, 353-361 43. Sivan G., N. Kedersha, O. Elroy-Stein (2007) Ribosomal slowdown mediates translational arrest during cellular division. Mol Cell Biol. 27, 6639-6646 44. Sivan G. and O. Elroy-Stein (2008) Translational control during cellular division. Cell cycle. Vol. 7, 741-744 45. Marash L., N. Liberman, S. Henis-Korenblit, G. Sivan, E. Reem, O. Elroy-Stein, A. Kimchi (2008) DAP5 promotes cap-independent translation of Bcl-2 and CDK1 to facilitate cell survival during mitosis. Mol Cell. 30, 447-459 46. Kantor L, D. Pinchasi, M. Mintz, Y. Hathout, A. Vanderver, O. Elroy-Stein (2008) A Point mutation in Translation Initiation Factor 2B Leads to a Continuous Hyper Stress State in Oligodendroglial-Derived Cells. PLoS ONE. 3(11):e3783. 47. Marom L., S. Hen, D. Pinchasi, J.A. Chekanova, D.A. Belostotsky, O. Elroy-Stein (2009). Diverse poly(A) binding proteins mediate internal translational initiation by a plant viral IRES. RNA Biol. 6(4), 446-454 48. Raveh-Amit H., A. Maissel, J. Poller, L. Marom, O. Elroy-Stein, M. Shapira, and E. Livneh (2009). Translational Control of Protein Kinase C by Two Upstream Open Reading Frames (uORFs). Mol Cell Biol. 29(22), 6140-6148 49. Geva M., Y. Cabilly, Y. Assaf, N. Mindroul, L. Marom, G. Raini, D. Pinchasi and O. ElroyStein. (2010). A mouse model for eIF2B-leukodystrophy reveals abnormal development of brain white matter. Brain. 133, 2448-2461 50. Horzinski, L., L. Kantor, A. Huyghe, R. Schiffmann, O. Elroy-Stein, O. Boespflug-Tanguy and A. Fogli (2010). Evaluation of the endoplasmic reticulum-stress response in eIF2Bmutated lymphocytes and lymphoblasts from CACH/VWM patients. BMC Neurology. 10, 94-101 51. Mor, E., Y. Cabilly, Y. Goldshmit, H. Zalts, S. Modai, L. Edry, O. Elroy-Stein, and N. Shomron (2011) Species-specific microRNA roles elucidated following astrocyte activation. Nucleic Acids Res. 39, 3710-3723. 52. Sivan G., Aviner R., and O. Elroy-Stein (2011) Mitotic modulation of translation elongation factor 1 leads to hindered tRNA delivery to ribosomes. J. Biol. Chem. 286 (32), 279277935 4 Orna Elroy-Stein, Ph.D. Tel Aviv University 53. Barhoom, S., J. Kaur, B. S. Cooperman, N. I. Smorodinsky, Z. Smilansky, M. Ehrlich, and O. Elroy-Stein (2011) Quantitative single cell monitoring of protein synthesis at subcellular resolution using fluorescently labeled tRNA. Nucleic Acids Res. 39:e129, July 27, 1-13 54. Marom, L., Ulitsky I., Cabilly Y., Shamir, R., and O. Elroy-Stein (2011) A point mutation in translation initiation factor eIF2B leads to function- and time- specific changes in brain gene expression. PLoS One, 3(11): e3783 55. Cabilly Y., Brabi M., Geva M., Marom L., Chetrit D, Ehrlich M., and O. Elroy-Stein (2012) Poor inflammatory response in eIF2B knock-in mice: Implications for the etiology of Vanishing White Matter diseas. PloS One 7(10):e46715 56. Aviner R., Geiger T., and O. Elroy-Stein (2013) Novel Proteomic Approach (PUNCH-P) Reveals Cell Cycle Specific Fluctuations in mRNA Translation. Genes Dev. 27, 1834-1844 Highlighted in: Nature Chemical Biology Miura, G. "Pack a PUNCH". Research Highlights, Nature Chemical Biology 9, 601 (2013) doi:10.1038/nchembio.1347 Published online 17 September 2013 SciBX: Science-Buisness eXchange (Nature.com Publication) Lou, K-J. "Puromycin-associated nascent chain proteomics (PUNCH-P) for monitoring protein synthesis" SciBX 6(35); doi:10.1038/scibx.2013.971 Published online 12 September 2013 57. Barhoom S., Farrell I., Shai B., Dahari D., Cooperman B.S., Smilansky Z., Elroy-Stein O., and M. Ehrlich. (2013) Dicodon monitoring of protein synthesis (DiCoMPS) reveals levels of synthesis of a viral protein in single cells. Nucleic Acids Res. Aug 21, 1-10. doi: 10.1093/nar/gkt686 58. Aviner R., Geiger T., and O. Elroy-Stein. (2014) Genome-wise identification and quantification of protein synthesis in cultured cells and whole tissues by puromycin-associated nascent chain proteomics (PUNCH-P). Nature Protocols 9(4), 751-760 59. Aviner R., Geiger T., and Elroy-Stein O. (2014) PUNCH-P for global translatome profiling: Methodology, insights and comparison to other techniques. Translation 1(1), eLocation ID: e27516 http://dx.doi.org/10.4161/trla.27516 CHAPTERS IN BOOKS 1. Leiman-Hurwitz, J., N. Dafni, L. Sherman, D. Levanon, Y. Bernstein, E. Danciger, O. Elroy-Stein, Y. Groner. Molecular structure and expression of the gene locus on chromosome 21 encoding the Cu/Zn-superoxide dismutase and its relevance to Down's syndrome. New York, Ann. New York Acad. Sci. Vol.450, 1985 (pp.133-156). 2. Groner, Y., N. Dafni, L. Sherman, D. Levanon, Y. Bernstein, E. Danciger, O. Elroy-Stein, A. Neer. Down syndrome and Alzheimer's disease: Are common genes from human chromosome 21 involved in both disorders? In: A. Fisher, I. Hanin and C. Lachman (eds.) Alzheimer's and Parkinson's Disease, New York, Plenum Publish. Co., 1986 (pp. 271-285). 5 Orna Elroy-Stein, Ph.D. Tel Aviv University 3. Groner, Y., L. Leiman-Hurwitz, N. Dafni, Y. Bernstein, L. Sherman, D. Levanon, E. Danciger, O. Elroy-Stein. The human CuZnSOD family: Architecture and expression of the chromosom 21- encoded functional gene and its related processed pseudogenes. In: G. Rotilio (ed.) Superoxide and superoxide dismutase in Chemistry, Biology and Medicine. New York, Elsevier Sci. Publish. 1986 (pp. 257-265). 4. Elroy-Stein, O., Y. Bernstein, K.B. Avraham, N. Dafni, D. Levanon, E. Danciger, A. Neer, Y. Groner. Overexpression of the human Cu/Zn- superoxide dismutase gene in transfected cells - Implication to Down's syndrome. In: A. Shahar et al. (eds.) Model systems in Neurotoxicology - Alternative Approaches to Animal Testing. Progress in Clinical and Biological Research. New York, Alan R. Liss, Inc., Vol.253, 1987 (pp.373-392). 5. Fuerst, T.R., O. Elroy-Stein, B. Moss. Translation enhancement of mRNA conferred by Encephalomyocarditis virus 5' sequence improves the performance of the Vaccinia virus\ Bacteriophage T7 hybrid expression system. In: F. Brown, R.M. Chanock, H.S. Ginsberg and R.A. Lerner (eds.) Modern Approaches to New Vaccines Including Prevention of AIDS. Vaccines 90. Cold Spring Harbor, Cold Spring Harbor Laboratory Press, 1990 (pp.151-155) 6. Elroy-Stein O. and B. Moss. Gene Expression using the Vaccinia virus/T7 RNA Polymerase Hybrid System. In: F.M. Ausubel, R. Brent, R.E. Kingston, D.D. Moore, J.G. Seidman, J.A. Smith, and K. Struhlm (eds.) Current Protocols in Molecular Biology. New York, John Wiley & Sons, Inc. Vol.2, 1992, Unit 16.19 7. Elroy-Stein O. and W. C. Merrick. (2007) Translation initiation by cellular ribosomal entry sites. In: J.W.B. Hershey, M.B. Mathews, N. Sonenberg (eds.) Translational Control in Biology and Medicine. Cold Spring Harbor Laboratory Press, New York. 8. Elroy-Stein O. and R. Schiffmann. (2015) Vanishing White Matter Disease. In: J.M. Pascual and R.N. Rosenberg (eds.) ‘Rosenberg’s Molecular and Genetic Basis of Neurological and Psychiatric Disease, Fifth Edition’ Elsevier Academic Press, San Diego PATENTS Moss Bernard, Fuerst Thomas,Elroy-Stein Orna. Rapid, versatile and simple system for expressing genes in eukaryotic cells. Patent number: 5135855, Issued on 04/08/1992, USA Moss Bernard, Elroy-Stein Orna Stable mammalian cell line expressing a bacteriophage RNA polymerase. Patent number: 5126251, Issued on 30/06/1992, USA Barhoom Sima, Marcelo Ehrlich, Elroy-Stein Orna Detection of Cellular Stress by Live Imaging of tRNA Localization Ramot ref. 2010022-00-00; Filed on 22/7/2010 6