Links: | 2019 | 2018 | 2017 | 2016 | 2015 | Selected Publications


Unisciences, publications:

ORCID (Open Researcher and Contributor ID): 0000-0003-4475-9476

Thomson Reuters Research ID: A-2265-2012




Cresto N, Gaillard MC, Gardier C, Gubinelli F, Diguet E, Bellet D, Legroux L, Mitja J, Auregan G, Guillermier M, Josephine C, Jan C, Dufour N, Joliot A, Hantraye P, Bonvento G, Déglon N, Bemelmans AP, Cambon K, Liot G, Brouillet E. (2019). The C-terminal domain of LRRK2 with the G2019S mutation is sufficient to produce neurodegeneration of dopaminergic neurons in vivo. Neurobiol. Dis. (In Press) doi: 10.1016/j.nbd.2019.104614.

Jollé, C., Déglon, N., Pythoud, C., Bouzier-Sore, A.-K., Pellerin, L. (2019). Development of efficient AAV2/DJ-based viral vectors to selectively downregulate the expression of neuronal or astrocytic target proteins in the rat central nervous system. Front. Mol. Neurosci. doi: 10.3389/fnmol.2019.00201

Merienne, N., Meunier, C, Schneider, A., Seguin, J. Nair, S.S., Rocher, A. B, Le Gras, S., Keime, C., Faull, R., Pellerin, L. Chatton, J.-Y, Néri, C., Merienne, K., Déglon, N. (2019). Cell-type-specific gene expression profiling in adult mouse brain reveals normal and disease-state signatures. Cell Reports 26, 2477–2493

Blessing, D., Vachey, G., Pythoud, C., Rey, M., Padrun, V., Wurm, F.M., Schneider, B.L.*, and Déglon, N.* (2019) *Equally contributed to this work. Scalable production of AAV vectors in orbotally shaken HEK293 cells. Mol. Ther Meth Clin. Dev. 13:14-26



Dujardin, S., Bégard, S., Caillierez, R., Lachaud, C., Carrier, S., Lieger, S., Gonzalez, J.A., Deramecourt, V., Déglon, N., Maurage, C.-A., Frosch, M.P., Hyman, B.T., Colin, M., and Buée, L. (2018). Different Tau species lead to heterogeneous Tau pathology propagation and misfolding. Acta Neuropathologica Communications 6: 132

Perriot, S., Mathias, A., Perriard, G., Canales, M., Jonkmans, N., Merienne, N., Meunier, C., El Kassar, L., Perrier, A.L., Laplaud, D.-A., Schluep, M., Déglon, N., Du Pasquier, R. Human Induced Pluripotent Stem Cell-Derived Astrocytes Are Differentially Activated by Multiple Sclerosis-Associated Cytokines. Stem Cell Reports 1(5):1199-1210

Petrelli, F., Dallérac, G., Pucci, L., Cali, C., Zehnder, T., Sultan, S., Lecca, S., Chicca, A., Ivanov, A., Asensio, C. Gundersen, V., Toni, N., Knott, G., Magara, F., Gertsch, J., Kirchhoff, F., Déglon, Giros, B., Edwards, R., Mothet J.-P., Bezzi, P. (2018). Dysfunction of homeostatic control of dopamine by astrocytes in the developing prefrontal cortex leads to cognitive impairments. Molecular Psychiatry (In Press) DOI: 10.1038/s41380-018-0226-y

Audrain, M., Souchet, B., Alves, S., Fol, R., Viode, A., Haddjeri, A., Tada, S., Orefice, N.S., Joséphine, C., Bemelmans, A.-P., Delzescaux, T., Déglon, N., Hantraye, P., Akwa, Y., Becher, F., Billard, J.-M., Potier, B., Dutar, P., Cartier, N. and Braudeau, J. (2017). βAPP Processing Drives Gradual Tau Pathology in an Age-Dependent Amyloid Rat Model of Alzheimer’s Disease. Cerebral Cortex 28: 3976-3993

Pircs, K., Petri, R., Madsen, S., Matusiak-Brückner, M., Vuono, R., Déglon, N., Barker, R.A. & Jakobsson, J. (2018). Huntingtin aggregation impairs autophagy leading to Argonaute-2 accumulation and global microRNA dysregulation. Cell Reports 6: 397-1406

Cheng, S., Tereshchenko, J., Zimmer, V., Vachey, G., Pythoud, C., Rey, M., Liefhebber, J., Raina, A., Streit, F., Mazur, A., Bähr, M., Konstantinova, P., Déglon, N., Kügler, S. (2018). Therapeutic efficacy of regulable GDNF expression for Huntington´s and Parkinson´s disease by a high-induction, background-free "GeneSwitch" vector. Exp. Neurol. 309: 79-90

Galvan, L., Francelle, L., Gaillard, M. C., de Longprez, L., Carrillo-de Sauvage, M. A., Liot, G., Cambon, K., Stimmer, L., Luccantoni, S., Flament, J., Valette, J., de Chaldee, M., Auregan, G., Guillermier, M., Josephine, C., Petit, F., Jan, C., Jarrige, M., Dufour, N., Bonvento, G., Humbert, S., Saudou, F., Hantraye, P., Merienne, K., Bemelmans, A. P., Perrier, A. L., Déglon, N., Brouillet, E. (2018). The striatal kinase DCLK3 produces neuroprotection against mutant huntingtin. Brain 141: 1434-1454.

Novati, A., Hentrich, T., Wassouf, Z., Weber, J.J., Yu-Taeger, L., Déglon, N., Nguyen, H.P., Schultze-Hentrich, J.M. (2018). Environment-dependent striatal gene expression in the BACHD rat model for Huntington’s disease. Sci. Rep. 8: 5803.

Vachey, G and Déglon, N. (2018). CRISPR/Cas9-mediated genome editing for Huntington' disease. Methods Mol Biol.1780: 463-481.

Blessing D, Déglon N, Schneider BL. (2018). Scalable production and Purification of Adeno-Associated viral Vcetors (AAV). Methods Mol Biol. 1850: 259-274.




Galan-Rodriguez B., Martin E., Brouillet E., Déglon N., Betuing, S. and Caboche J. (2017). Specific coupling of D2short receptor isoform to the Rho/Rock signaling pathway renders striatal neurons more vulnerable to mutant huntingtin. Eur. J. Neurochem. 45(1): 198-206

Schwab, L.C., Richetin, K., Barker, R.A. and Déglon, N. (2017). Formation of hippocampal mHTT aggregates leads to impaired spatial memory, hippocampal activation and adult neurogenesis. Neurobiol. Dis. 102:105-112

Mazuel, L., Blanc, J., LRepond, C., Bouchard, V., Raffard G., Déglon, N., Bonvento, G., Pellerin, L., and Bouzier-Sore A.-K. (2017). A neuronal MCT2 knockdown in the rat somatosensory cortex reduces both the NMR lactate signal and the BOLD response during whisker stimulation. PlosOne 12 (4): e0174990

Déglon N. (2017). From huntingtin gene to Huntington's disease-altering strategies. Chapter 9: pp.251-276. In Disease-Modifying Targets in Neurodegenerative Disorders: paving the way for disease-modifying therapies. Elsevier Academin Press. Ed. Baekelandt V. and Lobbestael, E.

Cambon, K., Virginie Zimmer, Martineau, S., Gaillard, M.C., Jarrige. M., Bugi, A., Miniarikova, J., Rey, M., Hassig, R., Dufour, N., Auregan G., Hantraye, P., Perrier, A. L. and Déglon, N. (2017). Pre-clinical evaluation of a lentiviral vector huntingtin silencing. Mol. Ther. Meth. Clin. Dev. 5: 259-276

Merienne, N., Vachey, G., de Longprez, L., Meunier, C., Zimmer, V., Perriard, G., Canales M., Mathias, A., Hergott, L, Beltraminelli, T., Maulet, A., Dequesne, T., Pythoud, C., Rey, M., Pellerin, L., Brouillet, E., Perrier, A., du Pasquier, R., Déglon, N. (2017). The self-inactivating KamiCas9 system for the editing of CNS disease genes. Cell Reports 20: 2980-2991

Miniarikova, J.,  Zimmer, V., Martier, M., Brouwers, C.C., Pythoud, C., Richetin, K., Rey,M.,  Lubelski, J., Evers, m.M., van Deventer, S.J.,  Petry, H.,  Déglon, N., and Konstantinova, P. (2017). AAV5-miHTT gene therapy demonstrates suppression of mutant huntingtin aggregation and neuronal dysfunction in a rat model of Huntington’s disease. Gene Ther. 24: 630-639



Vázquez-Manrique, R.P., Farina, F., Cambon, K., Sequedo, M.D., Parker, A.J., Millán, J.M., Weiss, A., Déglon, N., Neri, C. (2016). AMPK activation protects from neuronal dysfunction and striatal vulnerability across nematode, cellular and mouse models of Huntington's disease. Hum. Mol. Genet. 25: 1043-1058

Lopes C., Aubert S., Bourgois-Rocha, F., Barnat M., Rego A.C., Déglon N., Perrier A. and Humbert S. (2016). Dominant-negative effects of adult-onset huntingtin mutations alter the division of human embryonic stem cells-derived neural cells. PlosOne 11(2):e0148680

Audrain, M., Fol, R., Dutar, P., Potier, B., Billard, J.-M., Flament, J., Alves, S., Burlot, M.-A., Dufayet-Chaffaud, G., Bemelmans, A.P., Valette, J., Hantraye, P., Déglon, N., Cartier, N., Braudeau, J. (2016). Human-like APP processing in mouse hippocampus identifies early synaptic alterations as key events of Alzheimer’s disease onset. Molecular Neurodegeneration 11(1):5

Blessing, D. and Déglon, N. (2016). Adeno-associated virus and lentivirus vectors: a refined toolkit for the central nervous system. Curr. opin. Virol. 21: 61-66

Meunier, C., Merienne, N., Jolle, C., Déglon, N., Pellerin, L. (2016). Astrocytes Are Key But Indirect Contributors to the Development of the Symptomatology and Pathophysiology of Huntington’s Disease. Glia 64(11):1841-56



Francelle L, Galvan L, Gaillard MC, Petit F, Bernay B, Guillermier M, Bonvento G, Dufour N, Elalouf JM, Hantraye P, Déglon N, de Chaldée M, Brouillet E. (2015). The striatal long noncoding RNA Abhd11os is neuroprotective against an N-terminal fragment of mutant huntingtin in vivo. Neurobiol Aging. 36: 1601.e7-1601.e16

Francelle, L., Galvan, L., Gaillard, M.C., Guillermier, M., Houitte, D., Bonvento, G., Petit, F., Jan, C., Dufour, N., Hantraye, P., Elalouf, J.M., de Chaldée, M., Déglon, N., Brouillet, E. (2015). Loss of the thyroid hormone binding protein Crym renders striatal neurons more vulnerable to mutant huntingtin in Huntington’s disease. Hum. Mol. Genet. 24: 1563–1573

Ben Haim L, Ceyzériat K, Carrillo-de Sauvage MA, Aubry F, Auregan G, Guillermier M, Ruiz M, Petit F, Houitte D, Faivre E, Vandesquille M, Aron-Badin R, Dhenain M, Déglon N, Hantraye P, Brouillet E, Bonvento G, Escartin C. (2015). The JAK/STAT3 Pathway Is a Common Inducer of Astrocyte Reactivity in Alzheimer's and Huntington's Diseases. J Neurosci. 35:2817-29

Merienne, N. and Déglon, N. (2015). Approches de gene silencing pour le traitement de la maladie de Huntington. Médecine et Science. 31: 159-167

Deloulme, J.-C., Gory-Fauré, S., Mauconduit, F., Chauvet, S., Jonckheere, J., Boulan, B., Mire, E., Xue, J., Jany, M., Maucler, C., Deparis, A., Montigon, O., Daoust, A., Barbier, E.L., Bosc, C., Déglon, N., Brocard, J., Denarier, E., Lebrun, I., Pernet-Gallay, K., Vilgrain , I., Robinson, P.J., Lahrech , H., Mann, F., and Andrieux, A. (2015). Microtubule-Associated Protein 6 (MAP6) mediates neuronal connectivity through Semaphorin 3E-dependent signaling for axonal growth. Nat. Comm. 6:7246

Merienne, N., Delzor, A., Viret, A., Dufour, N., Rey, M., Hantraye, P., and Déglon, N. (2015). Gene transfer engineering for astrocyte-specific silencing in the CNS. Gene Ther. 22: 830-839

Quesseveur, G., Portal, B., Basile, J.-A., Ezan, P., Mathou, A., Halley, H., Leloup, C., Fioramonti, X., Déglon, N., Giaume, C., Rampon, C., Guiard, B.P. (2015). Attenuated levels of hippocampal connexin 43 and its phosphorylation correlate with antidepressant- and anxiolytic-like activities in mice. Front. Cell. Neurosci. 9: 490


Selected Publications

Feyeux, M., Bourgois-Rocha, F., Redfern, A., Giles, P., Lefort, N., Aubert, S., Bonnefond, C., Bugi, A., Déglon, N., Marta, R., Jones,L., Peschanski, M., Allen, N.D., and Perrier, A.L. (2012). Early transcriptional changes linked to naturally occurring Huntington’s disease mutations in human embryonic stem cells. Hum. Mol. Genet. 21: 3883-95

Drouet, V., Perrin, V., Hassig, R., Dufour N., Alves, S., Bonvento, G., Brouillet, E., Luthi-Carter, R., Hantraye, P. and Déglon N. (2009). Sustained effects of non-allele-specific huntingtin silencing. Ann. Neurol. 65: 276-285

Colin, A., Faideau, M., Dufour, N., Auregan, G., Hassig,R., Andrieu, T., Brouillet, E., Hantraye, P., Bonvento, G., and Déglon, N. (2009). Engineered lentiviral vector targeting astrocytes In vivo. Glia 57: 667-679

Runne , H., Régulier , E., Zala , D., Kuhn , A., Gokce , O., Sick , B., Aebischer , P., Déglon, N., and Luthi-Carter, L. (2008). Dysregulation of gene expression in primary neuron models of Huntington's disease shows that polyglutamine-related effects on the striatal transcriptome may not be dependent on brain circuitry. J. Neurosci. 28: 9723-9731

Benchoua, A., Trioulier, Y., Diguet , E., Malgorn, C., Gaillard , M.-C., Dufour, N., Elalouf , J.-M., Krajewski, S., Hantraye, P., Déglon , N. and Brouillet, E. (2008). Dopamine determines the vulnerability of striatal neurons to the N-terminal fragment of mutant huntingtin through the regulation of mitochondrial complex II. Hum. Mol. Genet. 17:1446-56

Palfi, S., Brouillet, E., Jarraya, B., Bloch, J., Jan, C., Shin, M., Condé, F., Li, X.-J., Aebischer, P., Hantraye, P., and Déglon N. (2007). Expression of an N-terminal fragment of mutated huntingtin in the lateral putamen is sufficient to produce abnormal movements in non-human primates. Mol. Ther. 15: 1444-1451

Zala, D., Benchoua, A., Brouillet, E., Perrin, V., Gaillard, M. C., Zurn, A. D., Aebischer, P. and Déglon, N. (2005). Progressive and selective striatal degeneration in primary neuronal cultures using lentiviral vector coding for a mutant huntingtin fragment. Neurobiol. Dis. 20: 785-798

de Almeida P.L., Ross C. A., Zala,D., Aebischer,P. and Déglon, N. (2002). Lentiviral-mediated delivery of mutant huntingtin in the striatum of rats induces a selective neuropathology modulated by polyglutamine repeat size, huntingtin expression levels and protein length. J. Neurosci. 22: 3473-3483

Kordower, J. H., Emborg, M., Bloch, J., Ma, S. Y., Chu, Y., Leventhal, L., McBride, J., Chen, E.-Y., Palfi, S., Roitberg, B. Z., Brown, D., Holden, J., Pyzalski, R., Taylor, M. D., Carvey, P., Ling, Z.D., Trono, D., Hantraye, P., Déglon, N. and Aebischer, P. (2000). Neurodegeneration prevented by lentiviral vector delivery of GDNF in primate models of Parkinson's disease. Science 290, 767-773

Déglon, N., Tseng, J.L., Bensadoun, J.-C., Zurn, A.D., Arsenijevic, Y., Pereira de Almeida, L., Zufferey, R., Trono and Aebischer, P. (2000). Self-inactivating lentiviral vectors with enhanced transgene expression as potential gene transfer system in Parkinson’s disease. Hum. Gene Ther. 11, 179-190



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