My Bibliography

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Lobato AG, Ortiz-Vega N, Canic T, Tao X, Bucan N, Ruan K, Rebelo AP, Schule R, Zuchner S, Syed S, Zhai RG. Loss of Fic causes progressive neurodegeneration in a Drosophila model of hereditary spastic paraplegia. Biochim Biophys Acta Mol Basis Dis. 2024 Oct;1870(7):167348. doi: 10.1016/j.bbadis.2024.167348. Epub 2024 Jul 8. PubMed PMID: 38986817; NIHMSID:NIHMS2010339.

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Lobato AG, Ortiz-Vega N, Zhu Y, Neupane D, Meier KK, Zhai RG. Copper enhances aggregational toxicity of mutant huntingtin in a Drosophila model of Huntington's Disease. Biochim Biophys Acta Mol Basis Dis. 2024 Jan;1870(1):166928. doi: 10.1016/j.bbadis.2023.166928. Epub 2023 Oct 28. PubMed PMID: 38660915; PubMed Central PMCID: PMC11046041.

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Ruan K, Perez GF, Liu J, Kubat M, Hofacker I, Wuchty S, Zhai RG. MicroRNA-Mediated Obstruction of Stem-loop Alternative Splicing (MIMOSAS): a global mechanism for the regulation of alternative splicing. bioRxiv. 2023 Jun 28;. doi: 10.1101/2023.04.14.536877. PubMed PMID: 37425843; PubMed Central PMCID: PMC10327045.

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Zhu Y, Lobato AG, Rebelo AP, Canic T, Ortiz-Vega N, Tao X, Syed S, Yanick C, Saporta M, Shy M, Perfetti R, Shendelman S, Züchner S, Zhai RG. Sorbitol reduction via govorestat ameliorates synaptic dysfunction and neurodegeneration in sorbitol dehydrogenase deficiency. JCI Insight. 2023 May 22;8(10). doi: 10.1172/jci.insight.164954. PubMed PMID: 37014713; PubMed Central PMCID: PMC10322690.

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Stewart TRM, Foley JR, Holbert CE, Khomutov MA, Rastkari N, Tao X, Khomutov AR, Zhai RG, Casero RA. Difluoromethylornithine rebalances aberrant polyamine ratios in Snyder-Robinson syndrome: mechanism of action and therapeutic potential. bioRxiv. 2023 Apr 10;. doi: 10.1101/2023.03.30.534977. PubMed PMID: 37034775; PubMed Central PMCID: PMC10081208.

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Tao X, Liu J, Diaz-Perez Z, Foley JR, Stewart TM, Casero RA Jr, Zhai RG. Reduction of Spermine Synthase Suppresses Tau Accumulation Through Autophagy Modulation in Tauopathy. bioRxiv. 2023 Mar 18;. doi: 10.1101/2023.03.17.533015. PubMed PMID: 36993333; PubMed Central PMCID: PMC10055309.

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Zhang S, Zhu Y, Lu J, Liu Z, Lobato AG, Zeng W, Liu J, Qiang J, Zeng S, Zhang Y, Liu C, Liu J, He Z, Zhai RG, Li D. Specific binding of Hsp27 and phosphorylated Tau mitigates abnormal Tau aggregation-induced pathology. Elife. 2022 Sep 1;11. doi: 10.7554/eLife.79898. PubMed PMID: 36048712; PubMed Central PMCID: PMC9436411.

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Tao X, Zhu Y, Diaz-Perez Z, Yu SH, Foley JR, Stewart TM, Casero RA Jr, Steet R, Zhai RG. Phenylbutyrate modulates polyamine acetylase and ameliorates Snyder-Robinson syndrome in a Drosophila model and patient cells. JCI Insight. 2022 Jul 8;7(13). doi: 10.1172/jci.insight.158457. PubMed PMID: 35801587; PubMed Central PMCID: PMC9310527.

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Zhu Y, Lobato AG, Zhai RG, Pinto M. Human Nmnat1 Promotes Autophagic Clearance of Amyloid Plaques in a Drosophila Model of Alzheimer's Disease. Front Aging Neurosci. 2022;14:852972. doi: 10.3389/fnagi.2022.852972. eCollection 2022. PubMed PMID: 35401143; PubMed Central PMCID: PMC8988035.

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Liu J, Tao X, Zhu Y, Li C, Ruan K, Diaz-Perez Z, Rai P, Wang H, Zhai RG. NMNAT promotes glioma growth through regulating post-translational modifications of P53 to inhibit apoptosis. Elife. 2021 Dec 17;10. doi: 10.7554/eLife.70046. PubMed PMID: 34919052; PubMed Central PMCID: PMC8683086.

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Tantak MP, Sekhar V, Tao X, Zhai RG, Phanstiel O 4th. Development of a Redox-Sensitive Spermine Prodrug for the Potential Treatment of Snyder Robinson Syndrome. J Med Chem. 2021 Nov 11;64(21):15593-15607. doi: 10.1021/acs.jmedchem.1c00419. Epub 2021 Oct 25. PubMed PMID: 34695351; PubMed Central PMCID: PMC9115777.

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Hu J, Liu J, Zhu Y, Diaz-Perez Z, Sheridan M, Royer H, Leibensperger R 3rd, Maizel D, Brand L, Popendorf KJ, Gaston CJ, Zhai RG. Exposure to Aerosolized Algal Toxins in South Florida Increases Short- and Long-Term Health Risk in Drosophila Model of Aging. Toxins (Basel). 2020 Dec 11;12(12). doi: 10.3390/toxins12120787. PubMed PMID: 33322328; PubMed Central PMCID: PMC7763642.

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Cortese A, Zhu Y, Rebelo AP, Negri S, Courel S, Abreu L, Bacon CJ, Bai Y, Bis-Brewer DM, Bugiardini E, Buglo E, Danzi MC, Feely SME, Athanasiou-Fragkouli A, Haridy NA, Isasi R, Khan A, Laurà M, Magri S, Pipis M, Pisciotta C, Powell E, Rossor AM, Saveri P, Sowden JE, Tozza S, Vandrovcova J, Dallman J, Grignani E, Marchioni E, Scherer SS, Tang B, Lin Z, Al-Ajmi A, Schüle R, Synofzik M, Maisonobe T, Stojkovic T, Auer-Grumbach M, Abdelhamed MA, Hamed SA, Zhang R, Manganelli F, Santoro L, Taroni F, Pareyson D, Houlden H, Herrmann DN, Reilly MM, Shy ME, Zhai RG, Zuchner S. Biallelic mutations in SORD cause a common and potentially treatable hereditary neuropathy with implications for diabetes. Nat Genet. 2020 May;52(5):473-481. doi: 10.1038/s41588-020-0615-4. Epub 2020 May 4. PubMed PMID: 32367058; PubMed Central PMCID: PMC8353599.

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Ma X, Zhu Y, Lu J, Xie J, Li C, Shin WS, Qiang J, Liu J, Dou S, Xiao Y, Wang C, Jia C, Long H, Yang J, Fang Y, Jiang L, Zhang Y, Zhang S, Zhai RG, Liu C, Li D. Nicotinamide mononucleotide adenylyltransferase uses its NAD(+) substrate-binding site to chaperone phosphorylated Tau. Elife. 2020 Apr 6;9. doi: 10.7554/eLife.51859. PubMed PMID: 32250733; PubMed Central PMCID: PMC7136026.

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Lukacs M, Gilley J, Zhu Y, Orsomando G, Angeletti C, Liu J, Yang X, Park J, Hopkin RJ, Coleman MP, Zhai RG, Stottmann RW. Severe biallelic loss-of-function mutations in nicotinamide mononucleotide adenylyltransferase 2 (NMNAT2) in two fetuses with fetal akinesia deformation sequence. Exp Neurol. 2019 Oct;320:112961. doi: 10.1016/j.expneurol.2019.112961. Epub 2019 May 25. PubMed PMID: 31136762; PubMed Central PMCID: PMC6708453.

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Feng H, Park JS, Zhai RG, Wilson ACC. microRNA-92a regulates the expression of aphid bacteriocyte-specific secreted protein 1. BMC Res Notes. 2019 Sep 30;12(1):638. doi: 10.1186/s13104-019-4665-6. PubMed PMID: 31564246; PubMed Central PMCID: PMC6767646.

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Park J, Zhu Y, Tao X, Brazill JM, Li C, Wuchty S, Zhai RG. MicroRNA miR-1002 Enhances NMNAT-Mediated Stress Response by Modulating Alternative Splicing. iScience. 2019 Sep 27;19:1048-1064. doi: 10.1016/j.isci.2019.08.052. Epub 2019 Aug 30. PubMed PMID: 31522116; PubMed Central PMCID: PMC6745518.

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Zhu Y, Li C, Tao X, Brazill JM, Park J, Diaz-Perez Z, Zhai RG. Nmnat restores neuronal integrity by neutralizing mutant Huntingtin aggregate-induced progressive toxicity. Proc Natl Acad Sci U S A. 2019 Sep 17;116(38):19165-19175. doi: 10.1073/pnas.1904563116. Epub 2019 Sep 4. PubMed PMID: 31484760; PubMed Central PMCID: PMC6754563.

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Zhu Y, Liu J, Park J, Rai P, Zhai RG. Subcellular compartmentalization of NAD(+) and its role in cancer: A sereNADe of metabolic melodies. Pharmacol Ther. 2019 Aug;200:27-41. doi: 10.1016/j.pharmthera.2019.04.002. Epub 2019 Apr 8. Review. PubMed PMID: 30974124; PubMed Central PMCID: PMC7010080.

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Li C, Bademci G, Subasioglu A, Diaz-Horta O, Zhu Y, Liu J, Mitchell TG, Abad C, Seyhan S, Duman D, Cengiz FB, Tokgoz-Yilmaz S, Blanton SH, Farooq A, Walz K, Zhai RG, Tekin M. Dysfunction of GRAP, encoding the GRB2-related adaptor protein, is linked to sensorineural hearing loss. Proc Natl Acad Sci U S A. 2019 Jan 22;116(4):1347-1352. doi: 10.1073/pnas.1810951116. Epub 2019 Jan 4. PubMed PMID: 30610177; PubMed Central PMCID: PMC6347722.

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Brazill JM, Zhu Y, Li C, Zhai RG. Quantitative Cell Biology of Neurodegeneration in Drosophila Through Unbiased Analysis of Fluorescently Tagged Proteins Using ImageJ. J Vis Exp. 2018 Aug 3;(138). doi: 10.3791/58041. PubMed PMID: 30124668; PubMed Central PMCID: PMC6126610.

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Brazill JM, Cruz B, Zhu Y, Zhai RG. Nmnat mitigates sensory dysfunction in a Drosophila model of paclitaxel-induced peripheral neuropathy. Dis Model Mech. 2018 Jun 12;11(6). doi: 10.1242/dmm.032938. PubMed PMID: 29716954; PubMed Central PMCID: PMC6031360.

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Li C, Brazill JM, Liu S, Bello C, Zhu Y, Morimoto M, Cascio L, Pauly R, Diaz-Perez Z, Malicdan MCV, Wang H, Boccuto L, Schwartz CE, Gahl WA, Boerkoel CF, Zhai RG. Spermine synthase deficiency causes lysosomal dysfunction and oxidative stress in models of Snyder-Robinson syndrome. Nat Commun. 2017 Nov 2;8(1):1257. doi: 10.1038/s41467-017-01289-7. PubMed PMID: 29097652; PubMed Central PMCID: PMC5668419.

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Brazill JM, Li C, Zhu Y, Zhai RG. NMNAT: It's an NAD(+) synthase… It's a chaperone… It's a neuroprotector. Curr Opin Genet Dev. 2017 Jun;44:156-162. doi: 10.1016/j.gde.2017.03.014. Epub 2017 Apr 23. Review. PubMed PMID: 28445802; PubMed Central PMCID: PMC5515290.

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Gall T, Valkanas E, Bello C, Markello T, Adams C, Bone WP, Brandt AJ, Brazill JM, Carmichael L, Davids M, Davis J, Diaz-Perez Z, Draper D, Elson J, Flynn ED, Godfrey R, Groden C, Hsieh CK, Fischer R, Golas GA, Guzman J, Huang Y, Kane MS, Lee E, Li C, Links AE, Maduro V, Malicdan MCV, Malik FS, Nehrebecky M, Park J, Pemberton P, Schaffer K, Simeonov D, Sincan M, Smedley D, Valivullah Z, Wahl C, Washington N, Wolfe LA, Xu K, Zhu Y, Gahl WA, Tifft CJ, Toro C, Adams DR, He M, Robinson PN, Haendel MA, Zhai RG, Boerkoel CF. Defining Disease, Diagnosis, and Translational Medicine within a Homeostatic Perturbation Paradigm: The National Institutes of Health Undiagnosed Diseases Program Experience. Front Med (Lausanne). 2017;4:62. doi: 10.3389/fmed.2017.00062. eCollection 2017. PubMed PMID: 28603714; PubMed Central PMCID: PMC5445140.

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Ruetenik AL, Ocampo A, Ruan K, Zhu Y, Li C, Zhai RG, Barrientos A. Attenuation of polyglutamine-induced toxicity by enhancement of mitochondrial OXPHOS in yeast and fly models of aging. Microb Cell. 2016 Jul 26;3(8):338-351. doi: 10.15698/mic2016.08.518. PubMed PMID: 28357370; PubMed Central PMCID: PMC5349013.

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Ruan K, Zhu Y, Li C, Brazill JM, Zhai RG. Alternative splicing of Drosophila Nmnat functions as a switch to enhance neuroprotection under stress. Nat Commun. 2015 Nov 30;6:10057. doi: 10.1038/ncomms10057. PubMed PMID: 26616331; PubMed Central PMCID: PMC4674693.

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Ali YO, Li-Kroeger D, Bellen HJ, Zhai RG, Lu HC. NMNATs, evolutionarily conserved neuronal maintenance factors. Trends Neurosci. 2013 Nov;36(11):632-40. doi: 10.1016/j.tins.2013.07.002. Epub 2013 Aug 20. Review. PubMed PMID: 23968695; PubMed Central PMCID: PMC3857727.

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Kitay BM, McCormack R, Wang Y, Tsoulfas P, Zhai RG. Mislocalization of neuronal mitochondria reveals regulation of Wallerian degeneration and NMNAT/WLD(S)-mediated axon protection independent of axonal mitochondria. Hum Mol Genet. 2013 Apr 15;22(8):1601-14. doi: 10.1093/hmg/ddt009. Epub 2013 Jan 11. PubMed PMID: 23314018; PubMed Central PMCID: PMC3657477.

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Wen Y, Zhai RG, Kim MD. The role of autophagy in Nmnat-mediated protection against hypoxia-induced dendrite degeneration. Mol Cell Neurosci. 2013 Jan;52:140-51. doi: 10.1016/j.mcn.2012.11.008. Epub 2012 Nov 15. PubMed PMID: 23159780; PubMed Central PMCID: PMC3540192.

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Zang S, Ali YO, Ruan K, Zhai RG. Nicotinamide mononucleotide adenylyltransferase maintains active zone structure by stabilizing Bruchpilot. EMBO Rep. 2013 Jan;14(1):87-94. doi: 10.1038/embor.2012.181. Epub 2012 Nov 16. PubMed PMID: 23154466; PubMed Central PMCID: PMC3537136.

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Ouyang H, Ali YO, Ravichandran M, Dong A, Qiu W, MacKenzie F, Dhe-Paganon S, Arrowsmith CH, Zhai RG. Protein aggregates are recruited to aggresome by histone deacetylase 6 via unanchored ubiquitin C termini. J Biol Chem. 2012 Jan 20;287(4):2317-27. doi: 10.1074/jbc.M111.273730. Epub 2011 Nov 8. PubMed PMID: 22069321; PubMed Central PMCID: PMC3268394.

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Ali YO, Ruan K, Zhai RG. NMNAT suppresses tau-induced neurodegeneration by promoting clearance of hyperphosphorylated tau oligomers in a Drosophila model of tauopathy. Hum Mol Genet. 2012 Jan 15;21(2):237-50. doi: 10.1093/hmg/ddr449. Epub 2011 Sep 30. PubMed PMID: 21965302; PubMed Central PMCID: PMC3276290.

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Ljungberg MC, Ali YO, Zhu J, Wu CS, Oka K, Zhai RG, Lu HC. CREB-activity and nmnat2 transcription are down-regulated prior to neurodegeneration, while NMNAT2 over-expression is neuroprotective, in a mouse model of human tauopathy. Hum Mol Genet. 2012 Jan 15;21(2):251-67. doi: 10.1093/hmg/ddr492. Epub 2011 Oct 25. PubMed PMID: 22027994; PubMed Central PMCID: PMC3276285.

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Wen Y, Parrish JZ, He R, Zhai RG, Kim MD. Nmnat exerts neuroprotective effects in dendrites and axons. Mol Cell Neurosci. 2011 Sep;48(1):1-8. doi: 10.1016/j.mcn.2011.05.002. Epub 2011 May 9. PubMed PMID: 21596138; PubMed Central PMCID: PMC3152617.

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Ali YO, McCormack R, Darr A, Zhai RG. Nicotinamide mononucleotide adenylyltransferase is a stress response protein regulated by the heat shock factor/hypoxia-inducible factor 1alpha pathway. J Biol Chem. 2011 May 27;286(21):19089-99. doi: 10.1074/jbc.M111.219295. Epub 2011 Apr 8. PubMed PMID: 21478149; PubMed Central PMCID: PMC3099722.

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Ali YO, Escala W, Ruan K, Zhai RG. Assaying locomotor, learning, and memory deficits in Drosophila models of neurodegeneration. J Vis Exp. 2011 Mar 11;(49). doi: 10.3791/2504. PubMed PMID: 21445036; PubMed Central PMCID: PMC3197301.

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Zhou X, Escala W, Papapetropoulos S, Zhai RG. β-N-methylamino-L-alanine induces neurological deficits and shortened life span in Drosophila. Toxins (Basel). 2010 Nov;2(11):2663-79. doi: 10.3390/toxins2112663. Epub 2010 Nov 3. PubMed PMID: 22069570; PubMed Central PMCID: PMC3153171.

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Ali YO, Kitay BM, Zhai RG. Dealing with misfolded proteins: examining the neuroprotective role of molecular chaperones in neurodegeneration. Molecules. 2010 Oct 8;15(10):6859-87. doi: 10.3390/molecules15106859. Review. PubMed PMID: 20938400; PubMed Central PMCID: PMC3133442.

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Zhai RG, Rizzi M, Garavaglia S. Nicotinamide/nicotinic acid mononucleotide adenylyltransferase, new insights into an ancient enzyme. Cell Mol Life Sci. 2009 Sep;66(17):2805-18. doi: 10.1007/s00018-009-0047-x. Epub 2009 May 16. Review. PubMed PMID: 19448972; PubMed Central PMCID: PMC11115848.

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Zhou X, Escala W, Papapetropoulos S, Bradley WG, Zhai RG. BMAA neurotoxicity in Drosophila. Amyotroph Lateral Scler. 2009;10 Suppl 2:61-6. doi: 10.3109/17482960903273445. PubMed PMID: 19929734.

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Zhai RG, Zhang F, Hiesinger PR, Cao Y, Haueter CM, Bellen HJ. NAD synthase NMNAT acts as a chaperone to protect against neurodegeneration. Nature. 2008 Apr 17;452(7189):887-91. doi: 10.1038/nature06721. Epub 2008 Mar 16. PubMed PMID: 18344983; PubMed Central PMCID: PMC3150538.

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Zhai RG, Cao Y, Hiesinger PR, Zhou Y, Mehta SQ, Schulze KL, Verstreken P, Bellen HJ. Drosophila NMNAT maintains neural integrity independent of its NAD synthesis activity. PLoS Biol. 2006 Nov;4(12):e416. doi: 10.1371/journal.pbio.0040416. PubMed PMID: 17132048; PubMed Central PMCID: PMC1665629.

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Hiesinger PR, Zhai RG, Zhou Y, Koh TW, Mehta SQ, Schulze KL, Cao Y, Verstreken P, Clandinin TR, Fischbach KF, Meinertzhagen IA, Bellen HJ. Activity-independent prespecification of synaptic partners in the visual map of Drosophila. Curr Biol. 2006 Sep 19;16(18):1835-43. doi: 10.1016/j.cub.2006.07.047. PubMed PMID: 16979562; PubMed Central PMCID: PMC3351197.

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Hiesinger PR, Fayyazuddin A, Mehta SQ, Rosenmund T, Schulze KL, Zhai RG, Verstreken P, Cao Y, Zhou Y, Kunz J, Bellen HJ. The v-ATPase V0 subunit a1 is required for a late step in synaptic vesicle exocytosis in Drosophila. Cell. 2005 May 20;121(4):607-620. doi: 10.1016/j.cell.2005.03.012. PubMed PMID: 15907473; PubMed Central PMCID: PMC3351201.

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Mehta SQ, Hiesinger PR, Beronja S, Zhai RG, Schulze KL, Verstreken P, Cao Y, Zhou Y, Tepass U, Crair MC, Bellen HJ. Mutations in Drosophila sec15 reveal a function in neuronal targeting for a subset of exocyst components. Neuron. 2005 Apr 21;46(2):219-32. doi: 10.1016/j.neuron.2005.02.029. PubMed PMID: 15848801.

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Zhai RG, Bellen HJ. Hauling t-SNAREs on the microtubule highway. Nat Cell Biol. 2004 Oct;6(10):918-9. doi: 10.1038/ncb1004-918. PubMed PMID: 15459718.

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Zhai RG, Bellen HJ. The architecture of the active zone in the presynaptic nerve terminal. Physiology (Bethesda). 2004 Oct;19:262-70. doi: 10.1152/physiol.00014.2004. Review. PubMed PMID: 15381754.

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Verstreken P, Koh TW, Schulze KL, Zhai RG, Hiesinger PR, Zhou Y, Mehta SQ, Cao Y, Roos J, Bellen HJ. Synaptojanin is recruited by endophilin to promote synaptic vesicle uncoating. Neuron. 2003 Nov 13;40(4):733-48. doi: 10.1016/s0896-6273(03)00644-5. PubMed PMID: 14622578.

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Zhai RG, Hiesinger PR, Koh TW, Verstreken P, Schulze KL, Cao Y, Jafar-Nejad H, Norga KK, Pan H, Bayat V, Greenbaum MP, Bellen HJ. Mapping Drosophila mutations with molecularly defined P element insertions. Proc Natl Acad Sci U S A. 2003 Sep 16;100(19):10860-5. doi: 10.1073/pnas.1832753100. Epub 2003 Sep 5. PubMed PMID: 12960394; PubMed Central PMCID: PMC196893.