Nicole Déglon

Publications | Mémoires et thèses

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151 publications

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Dysfunction of homeostatic control of dopamine by astrocytes in the developing prefrontal cortex leads to cognitive impairments.
Petrelli F., Dallérac G., Pucci L., Calì C., Zehnder T., Sultan S., Lecca S., Chicca A., Ivanov A., Asensio C.S. et al. Molecular psychiatry. Peer-reviewed.
A New Tool for In Vivo Study of Astrocyte Connexin 43 in Brain.
Droguerre M., Tsurugizawa T., Duchêne A., Portal B., Guiard B.P., Déglon N., Rouach N., Hamon M., Mouthon F., Ciobanu L. et al., 2019/12/04. Scientific reports, 9 (1) p. 18292. Peer-reviewed.
The C-terminal domain of LRRK2 with the G2019S mutation is sufficient to produce neurodegeneration of dopaminergic neurons in vivo.
Cresto N., Gaillard M.C., Gardier C., Gubinelli F., Diguet E., Bellet D., Legroux L., Mitja J., Auregan G., Guillermier M. et al., 2019/10/09. Neurobiology of disease p. 104614. Peer-reviewed.
Scalable Production of AAV Vectors in Orbitally Shaken HEK293 Cells.
Blessing D., Vachey G., Pythoud C., Rey M., Padrun V., Wurm F.M., Schneider B.L., Déglon N., 2019/06/14. Molecular therapy. Methods & clinical development, 13 pp. 14-26. Peer-reviewed.
Cell-Type-Specific Gene Expression Profiling in Adult Mouse Brain Reveals Normal and Disease-State Signatures.
Merienne N., Meunier C., Schneider A., Seguin J., Nair S.S., Rocher A.B., Le Gras S., Keime C., Faull R., Pellerin L. et al., 2019/02/26. Cell reports, 26 (9) pp. 2477-2493.e9. Peer-reviewed.
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.
Jollé C., Déglon N., Pythoud C., Bouzier-Sore A.K., Pellerin L., 2019. Frontiers in molecular neuroscience, 12 p. 201. Peer-reviewed.
Different tau species lead to heterogeneous tau pathology propagation and misfolding.
Dujardin S., Bégard S., Caillierez R., Lachaud C., Carrier S., Lieger S., Gonzalez J.A., Deramecourt V., Déglon N., Maurage C.A. et al., 2018/11/29. Acta neuropathologica communications, 6 (1) p. 132. Peer-reviewed.
Human Induced Pluripotent Stem Cell-Derived Astrocytes Are Differentially Activated by Multiple Sclerosis-Associated Cytokines.
Perriot S., Mathias A., Perriard G., Canales M., Jonkmans N., Merienne N., Meunier C., El Kassar L., Perrier A.L., Laplaud D.A. et al., 2018/11/13. Stem cell reports, 11 (5) pp. 1199-1210. Peer-reviewed.
Therapeutic efficacy of regulable GDNF expression for Huntington's and Parkinson's disease by a high-induction, background-free "GeneSwitch" vector.
Cheng S., Tereshchenko J., Zimmer V., Vachey G., Pythoud C., Rey M., Liefhebber J., Raina A., Streit F., Mazur A. et al., 2018/11. Experimental neurology, 309 pp. 79-90. Peer-reviewed.
βAPP Processing Drives Gradual Tau Pathology in an Age-Dependent Amyloid Rat Model of Alzheimer's Disease.
Audrain M., Souchet B., Alves S., Fol R., Viode A., Haddjeri A., Tada S., Orefice N.S., Joséphine C., Bemelmans A.P. et al., 2018/11/01. Cerebral cortex, 28 (11) pp. 3976-3993. Peer-reviewed.
Huntingtin Aggregation Impairs Autophagy, Leading to Argonaute-2 Accumulation and Global MicroRNA Dysregulation.
Pircs K., Petri R., Madsen S., Brattås P.L., Vuono R., Ottosson D.R., St-Amour I., Hersbach B.A., Matusiak-Brückner M., Lundh S.H. et al., 2018/08/07. Cell reports, 24 (6) pp. 1397-1406. Peer-reviewed.
The striatal kinase DCLK3 produces neuroprotection against mutant huntingtin.
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. et al., 2018/05/01. Brain, 141 (5) pp. 1434-1454. Peer-reviewed.
Environment-dependent striatal gene expression in the BACHD rat model for Huntington disease.
Novati A., Hentrich T., Wassouf Z., Weber J.J., Yu-Taeger L., Déglon N., Nguyen H.P., Schulze-Hentrich J.M., 2018/04/11. Scientific reports, 8 (1) p. 5803. Peer-reviewed.
CRISPR/Cas9-Mediated Genome Editing for Huntington's Disease.
Vachey G., Déglon N., 2018. Methods in molecular biology, 1780 pp. 463-481. Peer-reviewed.
Scalable Production and Purification of Adeno-Associated Viral Vectors (AAV).
Blessing D., Déglon N., Schneider B.L., 2018. Methods in molecular biology, 1850 pp. 259-274. Peer-reviewed.
AAV5-miHTT gene therapy demonstrates suppression of mutant huntingtin aggregation and neuronal dysfunction in a rat model of Huntington's disease.
Miniarikova J., Zimmer V., Martier R., Brouwers C.C., Pythoud C., Richetin K., Rey M., Lubelski J., Evers M.M., van Deventer S.J. et al., 2017/10. Gene therapy, 24 (10) pp. 630-639. Peer-reviewed.
The Self-Inactivating KamiCas9 System for the Editing of CNS Disease Genes.
Merienne N., Vachey G., de Longprez L., Meunier C., Zimmer V., Perriard G., Canales M., Mathias A., Herrgott L., Beltraminelli T. et al., 2017/09/19. Cell reports, 20 (12) pp. 2980-2991. Peer-reviewed.
Preclinical Evaluation of a Lentiviral Vector for Huntingtin Silencing.
Cambon K., Zimmer V., Martineau S., Gaillard M.C., Jarrige M., Bugi A., Miniarikova J., Rey M., Hassig R., Dufour N. et al., 2017/06/16. Molecular therapy. Methods & clinical development, 5 pp. 259-276. Peer-reviewed.
Formation of hippocampal mHTT aggregates leads to impaired spatial memory, hippocampal activation and adult neurogenesis.
Schwab L.C., Richetin K., Barker R.A., Déglon N., 2017/06. Neurobiology of disease, 102 pp. 105-112. Peer-reviewed.
A neuronal MCT2 knockdown in the rat somatosensory cortex reduces both the NMR lactate signal and the BOLD response during whisker stimulation.
Mazuel L., Blanc J., Repond C., Bouchaud V., Raffard G., Déglon N., Bonvento G., Pellerin L., Bouzier-Sore A.K., 2017. PloS one, 12 (4) pp. e0174990. Peer-reviewed.
Coupling of D2R Short but not D2R Long receptor isoform to the Rho/ROCK signaling pathway renders striatal neurons vulnerable to mutant huntingtin.
Galan-Rodriguez B., Martin E., Brouillet E., Déglon N., Betuing S., Caboche J., 2017/01. The European journal of neuroscience, 45 (1) pp. 198-206. Peer-reviewed.
From huntingtin gene to Huntington's disease-altering strategies
Déglon Nicole, 2017. pp. 251-276 dans Disease-Modifying Targets in Neurodegenerative Disorders, Elsevier.
Adeno-associated virus and lentivirus vectors: a refined toolkit for the central nervous system.
Blessing D., Déglon N., 2016/12. Current opinion in virology, 21 pp. 61-66. Peer-reviewed.
Astrocytes are key but indirect contributors to the development of the symptomatology and pathophysiology of Huntington's disease.
Meunier C., Merienne N., Jollé C., Déglon N., Pellerin L., 2016/11. Glia, 64 (11) pp. 1841-1856. Peer-reviewed.
Alzheimer's disease-like APP processing in wild-type mice identifies synaptic defects as initial steps of disease progression.
Audrain M., Fol R., Dutar P., Potier B., Billard J.M., Flament J., Alves S., Burlot M.A., Dufayet-Chaffaud G., Bemelmans A.P. et al., 2016/01/12. Molecular neurodegeneration, 11 p. 5. Peer-reviewed.
AMPK activation protects from neuronal dysfunction and vulnerability across nematode, cellular and mouse models of Huntington's disease.
Vázquez-Manrique R.P., Farina F., Cambon K., Dolores Sequedo M., Parker A.J., Millán J.M., Weiss A., Déglon N., Neri C., 2016. Human Molecular Genetics, 25 (6) pp. 1043-1058. Peer-reviewed.
Dominant-Negative Effects of Adult-Onset Huntingtin Mutations Alter the Division of Human Embryonic Stem Cells-Derived Neural Cells.
Lopes C., Aubert S., Bourgois-Rocha F., Barnat M., Rego A.C., Déglon N., Perrier A.L., Humbert S., 2016. Plos One, 11 (2) pp. e0148680. Peer-reviewed.
Loss of the thyroid hormone-binding protein Crym renders striatal neurons more vulnerable to mutant huntingtin in Huntington's disease.
Francelle L., Galvan L., Gaillard M.C., Guillermier M., Houitte D., Bonvento G., Petit F., Jan C., Dufour N., Hantraye P. et al., 2015/02. Human Molecular Genetics, 24 (6) pp. 1563-1573. Peer-reviewed.
Approches de gene silencing pour le traitement de la maladie de Huntington [Gene silencing approaches for the treatment of Huntington's disease].
Merienne N., Déglon N., 2015. Médecine Sciences : M/s, 31 (2) pp. 159-167. Peer-reviewed.
Attenuated Levels of Hippocampal Connexin 43 and its Phosphorylation Correlate with Antidepressant- and Anxiolytic-Like Activities in Mice.
Quesseveur G., Portal B., Basile J.A., Ezan P., Mathou A., Halley H., Leloup C., Fioramonti X., Déglon N., Giaume C. et al., 2015. Frontiers in Cellular Neuroscience, 9 p. 490. Peer-reviewed.
Gene transfer engineering for astrocyte-specific silencing in the CNS.
Merienne N., Delzor A., Viret A., Dufour N., Rey M., Hantraye P., Déglon N., 2015. Gene Therapy, 22 (10) pp. 830-839. Peer-reviewed.
Microtubule-associated protein 6 mediates neuronal connectivity through Semaphorin 3E-dependent signalling for axonal growth.
Deloulme J.C., Gory-Fauré S., Mauconduit F., Chauvet S., Jonckheere J., Boulan B., Mire E., Xue J., Jany M., Maucler C. et al., 2015. Nature Communications, 6 p. 7246. Peer-reviewed.
The JAK/STAT3 Pathway Is a Common Inducer of Astrocyte Reactivity in Alzheimer's and Huntington's Diseases.
Ben Haim L., Ceyzériat K., Carrillo-de Sauvage M.A., Aubry F., Auregan G., Guillermier M., Ruiz M., Petit F., Houitte D., Faivre E. et al., 2015. Journal of Neuroscience, 35 (6) pp. 2817-2829. Peer-reviewed.
The striatal long noncoding RNA Abhd11os is neuroprotective against an N-terminal fragment of mutant huntingtin in vivo.
Francelle L., Galvan L., Gaillard M.C., Petit F., Bernay B., Guillermier M., Bonvento G., Dufour N., Elalouf J.M., Hantraye P. et al., 2015. Neurobiology of Aging, 36 (3) pp. 1601.e7-1601.16. Peer-reviewed.
Allele-specific silencing of mutant huntingtin in rodent brain and human stem cells.
Drouet V., Ruiz M., Zala D., Feyeux M., Auregan G., Cambon K., Troquier L., Carpentier J., Aubert S., Merienne N. et al., 2014. Plos One, 9 (6) pp. e99341. Peer-reviewed.
Connexin 30 sets synaptic strength by controlling astroglial synapse invasion.
Pannasch U., Freche D., Dallérac G., Ghézali G., Escartin C., Ezan P., Cohen-Salmon M., Benchenane K., Abudara V., Dufour A. et al., 2014. Nature Neuroscience, 17 (4) pp. 549-558.
Lentiviral vectors in Huntington's disease research and therapy
Delzor A., Dufour N., Déglon N., 2014. pp. 193-220 dans Brambilla R. (eds.) Viral Vectors in Neurobiology and Brain Diseases chap. 11, Humana Press.
Neuron-to-neuron wild-type Tau protein transfer through a trans-synaptic mechanism: relevance to sporadic tauopathies.
Dujardin S., Lécolle K., Caillierez R., Bégard S., Zommer N., Lachaud C., Carrier S., Dufour N., Aurégan G., Winderickx J. et al., 2014. Acta Neuropathologica Communications, 2 (1) p. 14.
RNA interference mitigates motor and neuropathological deficits in a cerebellar mouse model of machado-joseph disease.
Nóbrega C., Nascimento-Ferreira I., Onofre I., Albuquerque D., Déglon N., Pereira de Almeida L., 2014. Plos One, 9 (8) pp. e100086. Peer-reviewed.
SET translocation is associated with increase in caspase cleaved amyloid precursor protein in CA1 of Alzheimer and Down syndrome patients.
Facchinetti P., Dorard E., Contremoulins V., Gaillard M.C., Déglon N., Sazdovitch V., Guihenneuc-Jouyaux C., Brouillet E., Duyckaerts C., Allinquant B., 2014. Neurobiology of Aging, 35 (5) pp. 958-968.
Glia: a new cellular target in the treatment of major depression.
Guiard B., 2013/10. Current drug targets, 14 (11) p. 1219.
A role of mitochondrial complex II defects in genetic models of Huntington's disease expressing N-terminal fragments of mutant huntingtin.
Damiano M., Diguet E., Malgorn C., D'Aurelio M., Galvan L., Petit F., Benhaim L., Guillermier M., Houitte D., Dufour N. et al., 2013. Human Molecular Genetics, 22 (19) pp. 3869-3882. Peer-reviewed.
BDNF overexpression in mouse hippocampal astrocytes promotes local neurogenesis and elicits anxiolytic-like activities.
Quesseveur G., David D.J., Gaillard M.C., Pla P., Wu M.V., Nguyen H.T., Nicolas V., Auregan G., David I., Dranovsky A. et al., 2013. Translational Psychiatry, 3 (e253) pp. 1-13.
Beclin 1 mitigates motor and neuropathological deficits in genetic mouse models of Machado-Joseph disease.
Nascimento-Ferreira I., Nóbrega C., Vasconcelos-Ferreira A., Onofre I., Albuquerque D., Aveleira C., Hirai H., Déglon N., Pereira de Almeida L., 2013. Brain, 136 (Pt 7) pp. 2173-2188. Peer-reviewed.
Efficient gene delivery and selective transduction of astrocytes in the mammalian brain using viral vectors.
Merienne N., Le Douce J., Faivre E., Déglon N., Bonvento G., 2013. Frontiers in Cellular Neuroscience, 7 p. 106.
Lentiviral delivery of the human wild-type tau protein mediates a slow and progressive neurodegenerative tau pathology in the rat brain.
Caillierez R., Bégard S., Lécolle K., Deramecourt V., Zommer N., Dujardin S., Loyens A., Dufour N., Aurégan G., Winderickx J. et al., 2013. Molecular Therapy, 21 (7) pp. 1358-1368.
Lentiviral vectors: a powerful tool to target astrocytes in vivo.
Delzor A., Escartin C., Déglon N., 2013. Current Drug Targets, 14 (11) pp. 1336-1346. Peer-reviewed.
Lentiviral-mediated gene transfer of siRNAs for the treatment of Huntington's disease.
Cambon K., Déglon N., 2013. Methods in Molecular Biology, 1010 pp. 95-109.
Overexpression of mutant ataxin-3 in mouse cerebellum induces ataxia and cerebellar neuropathology.
Nóbrega C., Nascimento-Ferreira I., Onofre I., Albuquerque D., Conceição M., Déglon N., de Almeida L.P., 2013. Cerebellum, 12 (4) pp. 441-455. Peer-reviewed.
Silencing mutant ataxin-3 rescues motor deficits and neuropathology in machado-joseph disease transgenic mice.
Nóbrega C., Nascimento-Ferreira I., Onofre I., Albuquerque D., Hirai H., Déglon N., de Almeida L.P., 2013. Plos One, 8 (e52396) pp. 1-11.
Calpastatin-mediated inhibition of calpains in the mouse brain prevents mutant ataxin 3 proteolysis, nuclear localization and aggregation, relieving Machado-Joseph disease.
Simões A.T., Gonçalves N., Koeppen A., Déglon N., Kügler S., Duarte C.B., Pereira de Almeida L., 2012. Brain, 135 (8) pp. 2428-2439.
Capucin does not modify the toxicity of a mutant Huntingtin fragment in vivo.
Galvan L., Lepejová N., Gaillard M.C., Malgorn C., Guillermier M., Houitte D., Bonvento G., Petit F., Dufour N., Héry P. et al., 2012. Neurobiology of Aging, 33 (8) pp. 1845.e5-1845.e6. Peer-reviewed.
Early transcriptional changes linked to naturally occurring Huntington's disease mutations in neural derivatives of human embryonic stem cells.
Feyeux M., Bourgois-Rocha F., Redfern A., Giles P., Lefort N., Aubert S., Bonnefond C., Bugi A., Ruiz M., Deglon N. et al., 2012. Human Molecular Genetics, 21 (17) pp. 3883-3895.
Lentiviral delivery of human full-length wild-type Tau protein mediates progressive spatio-temporal and cortico-cortical propagated neurofibrillary degeneration in rat brain
Colin M., Dujardin S., Lecolle K., Caillierez R., Begard S., Zommer N., Dufour N., Auregan G., Hantraye P., Deglon N. et al., 2012. pp. A46 dans Collaborative Congress of the European Society of Gene and Cell Therapy/French Society of Cell and Gene Therapy, Human Gene Therapy.
Restricted transgene expression in the brain with cell-type specific neuronal promoters.
Delzor A., Dufour N., Petit F., Guillermier M., Houitte D., Auregan G., Brouillet E., Hantraye P., Déglon N., 2012. Human Gene Therapy Methods, 23 (4) pp. 242-254. Peer-reviewed.
Toward a rescue of huntingtin transcripts by using a trans-splicing strategy
Le Hir M., Lorain S., Deglon N., Kirik D., Callander G., Voit T., Garcia L., 2012. pp. A132 dans Collaborative Congress of the European Society of Gene and Cell Therapy/French Society of Cell and Gene Therapy, Human Gene Therapy.
Toward the treatment of Huntington's disease with RNAi
Drouet V., Ruiz M., Zala D., Feyeux M., Auregan G., Cambon K., Aubert S., Bourgois-Rocha F., Hassig R., Dufour N. et al., 2012. pp. A22-A23 dans Collaborative Congress of the European Society of Gene and Cell Therapy French Society of Cell and Gene Therapy, Human Gene Therapy.
Viral-mediated overexpression of mutant huntingtin to model HD in various species.
Ruiz M., Déglon N., 2012. Neurobiology of Disease, 48 (2) pp. 202-211.
Mitogen- and stress-activated protein kinase 1-induced neuroprotection in Huntington's disease: role on chromatin remodeling at the PGC-1-alpha promoter.
Martin E., Betuing S., Pagès C., Cambon K., Auregan G., Deglon N., Roze E., Caboche J., 2011. Human Molecular Genetics, 20 (12) pp. 2422-2434.
Nuclear factor erythroid 2-related factor 2 facilitates neuronal glutathione synthesis by upregulating neuronal excitatory amino acid transporter 3 expression.
Escartin C., Won S.J., Malgorn C., Auregan G., Berman A.E., Chen P.C., Déglon N., Johnson J.A., Suh S.W., Swanson R.A., 2011. Journal of Neuroscience, 31 (20) pp. 7392-7401.
Overexpression of the autophagic beclin-1 protein clears mutant ataxin-3 and alleviates Machado-Joseph disease.
Nascimento-Ferreira I., Santos-Ferreira T., Sousa-Ferreira L., Auregan G., Onofre I., Alves S., Dufour N., Colomer Gould V.F., Koeppen A., Deglon N. et al., 2011. Brain, 134 (5) pp. 1400-1415.
In vivo expression of polyglutamine-expanded huntingtin by mouse striatal astrocytes impairs glutamate transport: a correlation with Huntington's disease subjects.
Faideau M., Kim J., Cormier K., Gilmore R., Welch M., Auregan G., Dufour N., Guillermier M., Brouillet E., Hantraye P. et al., 2010. Human Molecular Genetics, 19 (15) pp. 3053-3067.
Mitochondria in Huntington's disease.
Damiano M., Galvan L., Déglon N., Brouillet E., 2010. Biochimica et Biophysica Acta-Molecular Basis of Disease, 1802 (1) pp. 52-61. Peer-reviewed.
Silencing ataxin-3 mitigates degeneration in a rat model of Machado-Joseph disease: no role for wild-type ataxin-3?
Alves S., Nascimento-Ferreira I., Dufour N., Hassig R., Auregan G., Nóbrega C., Brouillet E., Hantraye P., Pedroso de Lima M.C., Déglon N. et al., 2010. Human Molecular Genetics, 19 (12) pp. 2380-2394.
Viral Vectors for in Vivo Gene Transfer
Thévenot E., Dufour N., Déglon N., 2010. pp. 1069-1096 dans Boisseau P., Houdy P., Lahmani M., European Materials Research Society. (eds.) Nanoscience : Nanobiotechnology and Nanobiology chap. 23, Springer.
Diminished hippocalcin expression in Huntington's disease brain does not account for increased striatal neuron vulnerability as assessed in primary neurons.
Rudinskiy N., Kaneko Y.A., Beesen A.A., Gokce O., Régulier E., Déglon N., Luthi-Carter R., 2009. Journal of Neurochemistry, 111 (2) pp. 460-472.
Engineered lentiviral vector targeting astrocytes in vivo.
Colin A., Faideau M., Dufour N., Auregan G., Hassig R., Andrieu T., Brouillet E., Hantraye P., Bonvento G., Déglon N., 2009. Glia, 57 (6) pp. 667-679.
Human alpha-iduronidase gene transfer mediated by adeno-associated virus types 1, 2, and 5 in the brain of nonhuman primates: vector diffusion and biodistribution.
Ciron C., Cressant A., Roux F., Raoul S., Cherel Y., Hantraye P., Déglon N., Schwartz B., Barkats M., Heard J.M. et al., 2009. Human Gene Therapy, 20 (4) pp. 350-360.
Implication of the JNK pathway in a rat model of Huntington's disease.
Perrin V., Dufour N., Raoul C., Hassig R., Brouillet E., Aebischer P., Luthi-Carter R., Déglon N., 2009. Experimental Neurology, 215 (1) pp. 191-200.
Normal aging modulates the neurotoxicity of mutant huntingtin.
Diguet E., Petit F., Escartin C., Cambon K., Bizat N., Dufour N., Hantraye P., Déglon N., Brouillet E., 2009. Plos One, 4 (2) pp. e4637.
Positron emission tomography imaging demonstrates correlation between behavioral recovery and correction of dopamine neurotransmission after gene therapy.
Leriche L., Björklund T., Breysse N., Besret L., Grégoire M.C., Carlsson T., Dollé F., Mandel R.J., Déglon N., Hantraye P. et al., 2009. Journal of Neuroscience, 29 (5) pp. 1544-1553.
Sustained effects of nonallele-specific Huntingtin silencing.
Drouet V., Perrin V., Hassig R., Dufour N., Auregan G., Alves S., Bonvento G., Brouillet E., Luthi-Carter R., Hantraye P. et al., 2009. Annals of Neurology, 65 (3) pp. 276-285.
Allele-specific RNA silencing of mutant ataxin-3 mediates neuroprotection in a rat model of Machado-Joseph disease.
Alves S., Nascimento-Ferreira I., Auregan G., Hassig R., Dufour N., Brouillet E., Pedroso de Lima M.C., Hantraye P., Pereira de Almeida L., Déglon N., 2008. Plos One, 3 (10) pp. e3341.
Applications of lentiviral vectors for biology and gene therapy of neurological disorders.
Lundberg C., Björklund T., Carlsson T., Jakobsson J., Hantraye P., Déglon N., Kirik D., 2008. Current Gene Therapy, 8 (6) pp. 461-473.
Dopamine determines the vulnerability of striatal neurons to the N-terminal fragment of mutant huntingtin through the regulation of mitochondrial complex II.
Benchoua A., Trioulier Y., Diguet E., Malgorn C., Gaillard M.C., Dufour N., Elalouf J.M., Krajewski S., Hantraye P., Déglon N. et al., 2008. Human Molecular Genetics, 17 (10) pp. 1446-1456.
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.
Runne H., Régulier E., Kuhn A., Zala D., Gokce O., Perrin V., Sick B., Aebischer P., Déglon N., Luthi-Carter R., 2008. Journal of Neuroscience, 28 (39) pp. 9723-9731.
Haloperidol protects striatal neurons from dysfunction induced by mutated huntingtin in vivo.
Charvin D., Roze E., Perrin V., Deyts C., Betuing S., Pagès C., Régulier E., Luthi-Carter R., Brouillet E., Déglon N. et al., 2008. Neurobiology of Disease, 29 (1) pp. 22-29.
Human and simian immunodeficiency viruses deregulate early hematopoiesis through a Nef/PPARgamma/STAT5 signaling pathway in macaques.
Prost S., Le Dantec M., Augé S., Le Grand R., Derdouch S., Auregan G., Déglon N., Relouzat F., Aubertin A.M., Maillere B. et al., 2008. Journal of Clinical Investigation, 118 (5) pp. 1765-1775.
Striatal and nigral pathology in a lentiviral rat model of Machado-Joseph disease.
Alves S., Régulier E., Nascimento-Ferreira I., Hassig R., Dufour N., Koeppen A., Carvalho A.L., Simões S., de Lima M.C., Brouillet E. et al., 2008. Human Molecular Genetics, 17 (14) pp. 2071-2083.
Vers un nouveau rôle du récepteur PPAR-gamma dans le syndrome d'immunodéficience acquise et les hémopathies chez l'homme. [Nef and PPAR-gamma interact to suppress Stat5 expression in CD34+ progenitors from infected macaques].
Prost S., Le Dantec M., Augé S., Le Grand R., Derdouch S., Auregan G., Déglon N., Relouzat F., Aubertin A.M., Maillere B. et al., 2008. Médecine Sciences : M/S, 24 (5) pp. 551-553.
Activation of astrocytes by CNTF induces metabolic plasticity and increases resistance to metabolic insults.
Escartin C., Pierre K., Colin A., Brouillet E., Delzescaux T., Guillermier M., Dhenain M., Déglon N., Hantraye P., Pellerin L. et al., 2007/07. Journal of Neuroscience, 27 (27) pp. 7094-7104.
Expression of mutated huntingtin fragment in the putamen is sufficient to produce abnormal movement in non-human primates.
Palfi S., Brouillet E., Jarraya B., Bloch J., Jan C., Shin M., Condé F., Li X.J., Aebischer P., Hantraye P. et al., 2007. Molecular Therapy, 15 (8) pp. 1444-1451. Peer-reviewed.
Les vecteurs viraux pour le transfert de genes in vivo
Thévenot E., Dufour N., Déglon N., 2007. pp. 1055-1081 dans Lahmani M, Boisseau P., Houdy P. (eds.) Les Nanosciences. Tome 3: Nanobiotechnologies et Nanobiologie chap. 23, Belin.
Metabolic correction in oligodendrocytes derived from metachromatic leukodystrophy mouse model by using encapsulated recombinant myoblasts.
Consiglio A., Martino S., Dolcetta D., Cusella G., Conese M., Marchesini S., Benaglia G., Wrabetz L., Orlacchio A., Déglon N. et al., 2007. Journal of the Neurological Sciences, 255 (1-2) pp. 7-16.
Neuroprotection by Hsp104 and Hsp27 in lentiviral-based rat models of Huntington's disease.
Perrin V., Régulier E., Abbas-Terki T., Hassig R., Brouillet E., Aebischer P., Luthi-Carter R., Déglon N., 2007. Molecular Therapy, 15 (5) pp. 903-911.
Transplants of CNTF-producing cells for the treatment of Huntington's disease
Escartin C., Hantraye P., Déglon N., 2007. pp. 385-398 dans Emerich D.F., Halberstadt C. (eds.) Cellular Transplants: From Lab to Clinic, Academic Press.
CA150 expression delays striatal cell death in overexpression and knock-in conditions for mutant huntingtin neurotoxicity.
Arango M., Holbert S., Zala D., Brouillet E., Pearson J., Régulier E., Thakur A.K., Aebischer P., Wetzel R., Déglon N. et al., 2006. Journal of Neuroscience, 26 (17) pp. 4649-4659.
Ciliary neurotrophic factor activates astrocytes, redistributes their glutamate transporters GLAST and GLT-1 to raft microdomains, and improves glutamate handling in vivo.
Escartin C., Brouillet E., Gubellini P., Trioulier Y., Jacquard C., Smadja C., Knott G.W., Kerkerian-Le Goff L., Déglon N., Hantraye P. et al., 2006. Journal of Neuroscience, 26 (22) pp. 5978-5989.
Inhibition of calcineurin by FK506 protects against polyglutamine-huntingtin toxicity through an increase of huntingtin phosphorylation at S421.
Pardo R., Colin E., Régulier E., Aebischer P., Déglon N., Humbert S., Saudou F., 2006. Journal of Neuroscience, 26 (5) pp. 1635-1645.
Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin.
Benchoua A., Trioulier Y., Zala D., Gaillard M.C., Lefort N., Dufour N., Saudou F., Elalouf J.M., Hirsch E., Hantraye P. et al., 2006. Molecular Biology of the Cell, 17 (4) pp. 1652-1663.
Lentivirus-mediated expression of glutathione peroxidase: neuroprotection in murine models of Parkinson's disease
Ridet J. L., Bensadoun J. C., Deglon N., Aebischer P., Zurn A. D., 2006/01. Neurobiology of Disease, 21 (1) pp. 29-34.
Akt is altered in an animal model of Huntington's disease and in patients.
Colin E., Régulier E., Perrin V., Dürr A., Brice A., Aebischer P., Déglon N., Humbert S., Saudou F., 2005. European Journal of Neuroscience, 21 (6) pp. 1478-1488.
Minocycline in phenotypic models of Huntington's disease.
Bantubungi K., Jacquard C., Greco A., Pintor A., Chtarto A., Tai K., Galas M.C., Tenenbaum L., Déglon N., Popoli P. et al., 2005. Neurobiology of Disease, 18 (1) pp. 206-217. Peer-reviewed.
Progressive and selective striatal degeneration in primary neuronal cultures using lentiviral vector coding for a mutant huntingtin fragment.
Zala D., Benchoua A., Brouillet E., Perrin V., Gaillard M.C., Zurn A.D., Aebischer P., Déglon N., 2005. Neurobiology of Disease, 20 (3) pp. 785-798.
Viral vectors as tools to model and treat neurodegenerative disorders.
Déglon N., Hantraye P., 2005. Journal of Gene Medicine, 7 (5) pp. 530-539.
Encapsulated GDNF-producing C2C12 cells for Parkinson's disease: a pre-clinical study in chronic MPTP-treated baboons.
Kishima H., Poyot T., Bloch J., Dauguet J., Condé F., Dollé F., Hinnen F., Pralong W., Palfi S., Déglon N. et al., 2004. Neurobiology of Disease, 16 (2) pp. 428-439. Peer-reviewed.
Lentiviral nigral delivery of GDNF does not prevent neurodegeneration in a genetic rat model of Parkinson's disease.
Lo Bianco C., Déglon N., Pralong W., Aebischer P., 2004. Neurobiology of Disease, 17 (2) pp. 283-289.
Lentiviral-mediated gene transfer to model triplet repeat disorders.
Régulier E., Zala D., Aebischer P., Déglon N., 2004. Methods in Molecular Biology, 277 pp. 199-213.
Local GDNF expression mediated by lentiviral vector protects facial nerve motoneurons but not spinal motoneurons in SOD1(G93A) transgenic mice.
Guillot S., Azzouz M., Déglon N., Zurn A., Aebischer P., 2004. Neurobiology of Disease, 16 (1) pp. 139-149.
Long-term lentiviral-mediated expression of ciliary neurotrophic factor in the striatum of Huntington's disease transgenic mice.
Zala D., Bensadoun J.C., Pereira de Almeida L., Leavitt B.R., Gutekunst C.A., Aebischer P., Hayden M.R., Déglon N., 2004. Experimental Neurology, 185 (1) pp. 26-35.
Neuroprotective gene therapy for Huntington's disease, using polymer-encapsulated cells engineered to secrete human ciliary neurotrophic factor: results of a phase I study.
Bloch J., Bachoud-Lévi A.C., Déglon N., Lefaucheur J.P., Winkel L., Palfi S., Nguyen J.P., Bourdet C., Gaura V., Remy P. et al., 2004. Human Gene Therapy, 15 (10) pp. 968-975.
Survival of encapsulated human primary fibroblasts and erythropoietin expression under xenogeneic conditions.
Schwenter F., Schneider B.L., Pralong W.F., Déglon N., Aebischer P., 2004. Human Gene Therapy, 15 (7) pp. 669-680.
Activity analysis of housekeeping promoters using self-inactivating lentiviral vector delivery into the mouse retina.
Kostic C., Chiodini F., Salmon P., Wiznerowicz M., Deglon N., Hornfeld D., Trono D., Aebischer P., Schorderet D.F., Munier F.L. et al., 2003. Gene Therapy, 10 (9) pp. 818-821.
Delivery of ciliary neurotrophic factor via lentiviral-mediated transfer protects axotomized retinal ganglion cells for an extended period of time.
van Adel B.A., Kostic C., Déglon N., Ball A.K., Arsenijevic Y., 2003. Human Gene Therapy, 14 (2) pp. 103-115.
Early and reversible neuropathology induced by tetracycline-regulated lentiviral overexpression of mutant huntingtin in rat striatum.
Régulier E., Trottier Y., Perrin V., Aebischer P., Déglon N., 2003. Human Molecular Genetics, 12 (21) pp. 2827-2836.
French gene therapy group reports on the adverse event in a clinical trial of gene therapy for X-linked severe combined immune deficiency (X-SCID). Position statement from the European Society of Gene Therapy.
European Society of Gene Therapy, 2003. Journal of Gene Medicine, 5 (1) pp. 82-84.
Multiply attenuated, self-inactivating lentiviral vectors efficiently deliver and express genes for extended periods of time in adult rat cardiomyocytes in vivo.
Fleury S., Simeoni E., Zuppinger C., Déglon N., von Segesser L.K., Kappenberger L., Vassalli G., 2003. Circulation, 107 (18) pp. 2375-2382.
Optimization of human erythropoietin secretion from MLV-infected human primary fibroblasts used for encapsulated cell therapy.
Schwenter F., Déglon N., Aebischer P., 2003. Journal of Gene Medicine, 5 (3) pp. 246-257.
Presence of Gal-alpha1,3Gal epitope on xenogeneic lines: implications for cellular gene therapy based on the encapsulation technology.
Déglon N., Aubert V., Spertini F., Winkel L., Aebischer P., 2003. Xenotransplantation, 10 (3) pp. 204-213.
alpha -Synucleinopathy and selective dopaminergic neuron loss in a rat lentiviral-based model of Parkinson's disease.
Lo Bianco C., Ridet J.L., Schneider B.L., Deglon N., Aebischer P., 2002. Proceedings of the National Academy of Sciences of the United States of America, 99 (16) pp. 10813-10818.
Delivery of erythropoietin by encapsulated myoblasts in a genetic model of severe anemia.
Rinsch C., Dupraz P., Schneider B.L., Déglon N., Maxwell P.H., Ratcliffe P.J., Aebischer P., 2002. Kidney International, 62 (4) pp. 1395-1401.
Dose-dependent neuroprotective effect of ciliary neurotrophic factor delivered via tetracycline-regulated lentiviral vectors in the quinolinic acid rat model of Huntington's disease.
Régulier E., Pereira de Almeida L., Sommer B., Aebischer P., Déglon N., 2002. Human Gene Therapy, 13 (16) pp. 1981-1990.
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.
de Almeida L.P., Ross C.A., Zala D., Aebischer P., Déglon N., 2002. Journal of Neuroscience, 22 (9) pp. 3473-3483.
Lentiviral-mediated RNA interference.
Abbas-Terki T., Blanco-Bose W., Déglon N., Pralong W., Aebischer P., 2002. Human Gene Therapy, 13 (18) pp. 2197-2201.
Lentivirally delivered glial cell line-derived neurotrophic factor increases the number of striatal dopaminergic neurons in primate models of nigrostriatal degeneration.
Palfi S., Leventhal L., Chu Y., Ma S.Y., Emborg M., Bakay R., Déglon N., Hantraye P., Aebischer P., Kordower J.H., 2002. Journal of Neuroscience, 22 (12) pp. 4942-4954.
Lentiviruses as vectors for CNS diseases.
Déglon N., Aebischer P., 2002. Current Topics in Microbiology and Immunology, 261 pp. 191-209.
Long-term doxycycline-regulated secretion of erythropoietin by encapsulated myoblasts.
Sommer B., Rinsch C., Payen E., Dalle B., Schneider B., Déglon N., Henri A., Beuzard Y., Aebischer P., 2002. Molecular Therapy, 6 (2) pp. 155-161.
Neurospheres modified to produce glial cell line-derived neurotrophic factor increase the survival of transplanted dopamine neurons.
Ostenfeld T., Tai Y.T., Martin P., Déglon N., Aebischer P., Svendsen C.N., 2002. Journal of Neuroscience Research, 69 (6) pp. 955-965.
Seizure suppression by adenosine-releasing cells is independent of seizure frequency.
Boison D., Huber A., Padrun V., Déglon N., Aebischer P., Möhler H., 2002. Epilepsia, 43 (8) pp. 788-796.
Isolation of multipotent neural precursors residing in the cortex of the adult human brain
Arsenijevic Y., Villemure J. G., Brunet J. F., Bloch J. J., Deglon N., Kostic C., Zurn A., Aebischer P., 2001/07. Experimental Neurology, 170 (1) pp. 48-62. Peer-reviewed.
Gene transfer into neurons from hippocampal slices: comparison of recombinant Semliki Forest Virus, adenovirus, adeno-associated virus, lentivirus, and measles virus.
Ehrengruber M.U., Hennou S., Büeler H., Naim H.Y., Déglon N., Lundstrom K., 2001. Molecular and Cellular Neurosciences, 17 (5) pp. 855-871.
Grafts of adenosine-releasing cells suppress seizures in kindling epilepsy.
Huber A., Padrun V., Déglon N., Aebischer P., Möhler H., Boison D., 2001. Proceedings of the National Academy of Sciences of the United States of America, 98 (13) pp. 7611-7616.
Neuroprotective effect of a CNTF-expressing lentiviral vector in the quinolinic acid rat model of Huntington's disease.
de Almeida L.P., Zala D., Aebischer P., Déglon N., 2001. Neurobiology of Disease, 8 (3) pp. 433-446.
Neuroprotective effect of interleukin-6 and IL6/IL6R chimera in the quinolinic acid rat model of Huntington's syndrome.
Bensadoun J.C., de Almeida L.P., Dréano M., Aebischer P., Déglon N., 2001. European Journal of Neuroscience, 14 (11) pp. 1753-1761.
Complete and long-term rescue of lesioned adult motoneurons by lentiviral-mediated expression of glial cell line-derived neurotrophic factor in the facial nucleus.
Hottinger A.F., Azzouz M., Déglon N., Aebischer P., Zurn A.D., 2000/08. Journal of Neuroscience, 20 (15) pp. 5587-5593.
Lentiviral vectors as a gene delivery system in the mouse midbrain: cellular and behavioral improvements in a 6-OHDA model of Parkinson's disease using GDNF
Bensadoun J. C., Deglon N., Tseng J. L., Ridet J. L., Zurn A. D., Aebischer P., 2000/07. Experimental Neurology, 164 (1) pp. 15-24.
Efficient gene transfer and expression of biologically active glial cell line-derived neurotrophic factor in rat motoneurons transduced wit lentiviral vectors.
Cisterni C., Henderson C.E., Aebischer P., Pettmann B., Déglon N., 2000. Journal of Neurochemistry, 74 (5) pp. 1820-1828. Peer-reviewed.
Encapsulation de cellules génétiquement modifiées: applications dans le système nerveux central.
Déglon N., Aebischer P., 2000. pp. 293-301 dans Thérapie Génique, Ed. Tec et Doc / Lavoisier.
Gene transfer techniques for the delivery of GDNF in Parkinson's disease.
Ridet J.L., Déglon N., Aebischer P., 2000. Novartis Foundation Symposium, 231 pp. 202-219, 302-306.
Neurodegeneration prevented by lentiviral vector delivery of GDNF in primate models of Parkinson's disease.
Kordower J.H., Emborg M.E., Bloch J., Ma S.Y., Chu Y., Leventhal L., McBride J., Chen E.Y., Palfi S., Roitberg B.Z. et al., 2000. Science, 290 (5492) pp. 767-773. Peer-reviewed.
Neuroprotective gene therapy for Huntington's disease using a polymer encapsulated BHK cell line engineered to secrete human CNTF.
Bachoud-Lévi A.C., Déglon N., Nguyen J.P., Bloch J., Bourdet C., Winkel L., Rémy P., Goddard M., Lefaucheur J.P., Brugières P. et al., 2000. Human Gene Therapy, 11 (12) pp. 1723-1729. Peer-reviewed.
Restoration of cognitive and motor functions by ciliary neurotrophic factor in a primate model of Huntington's disease.
Mittoux V., Joseph J.M., Conde F., Palfi S., Dautry C., Poyot T., Bloch J., Deglon N., Ouary S., Nimchinsky E.A. et al., 2000. Human Gene Therapy, 11 (8) pp. 1177-1187. Peer-reviewed.
Self-inactivating lentiviral vectors with enhanced transgene expression as potential gene transfer system in Parkinson's disease.
Déglon N., Tseng J.L., Bensadoun J.C., Zurn A.D., Arsenijevic Y., Pereira de Almeida L., Zufferey R., Trono D., Aebischer P., 2000. Human Gene Therapy, 11 (1) pp. 179-190.
Cellular xenotransplantation.
Aebischer P., Hottinger A.F., Déglon N., 1999. Nature Medicine, 5 (8) p. 852.
Improvement of mouse beta-thalassemia upon erythropoietin delivery by encapsulated myoblasts.
Dalle B., Payen E., Regulier E., Deglon N., Rouyer-Fessard P., Beuzard Y., Aebischer P., 1999. Gene Therapy, 6 (2) pp. 157-161.
Lentiviral gene transfer to the nonhuman primate brain.
Kordower J.H., Bloch J., Ma S.Y., Chu Y., Palfi S., Roitberg B.Z., Emborg M., Hantraye P., Déglon N., Aebischer P., 1999. Experimental neurology, 160 (1) pp. 1-16. Peer-reviewed.
A gene therapy approach for the treatment of amyotrophic lateral sclerosis and Parkinson's disease.
Zurn A.D., Tseng J.L., Déglon N., Joseph J.M., Aebischer P., 1998. Advances in Pharmacology, 42 pp. 929-931.
Continuous delivery of human and mouse erythropoietin in mice by genetically engineered polymer encapsulated myoblasts.
Régulier E., Schneider B.L., Déglon N., Beuzard Y., Aebischer P., 1998. Gene Therapy, 5 (8) pp. 1014-1022.
A gene therapy approach to regulated delivery of erythropoietin as a function of oxygen tension.
Rinsch C., Régulier E., Déglon N., Dalle B., Beuzard Y., Aebischer P., 1997. Human Gene Therapy, 8 (16) pp. 1881-1889.
Gene therapy for amyotrophic lateral sclerosis (ALS) using a polymer encapsulated xenogenic cell line engineered to secrete hCNTF.
Aebischer P., Pochon N.A., Heyd B., Deglon N., Joseph J.M., Zurn A.D., Baetge E.E., Hammang J.P., Goddard M., Lysaght M. et al., 1996/05. Human Gene Therapy, 7 (7) pp. 851-860. Peer-reviewed.
Central nervous system delivery of recombinant ciliary neurotrophic factor by polymer encapsulated differentiated C2C12 myoblasts.
Déglon N., Heyd B., Tan S.A., Joseph J.M., Zurn A.D., Aebischer P., 1996. Human Gene Therapy, 7 (17) pp. 2135-2146.
Intrathecal delivery of CNTF using encapsulated genetically modified xenogeneic cells in amyotrophic lateral sclerosis patients.
Aebischer P., Schluep M., Déglon N., Joseph J.M., Hirt L., Heyd B., Goddard M., Hammang J.P., Zurn A.D., Kato A.C. et al., 1996. Nature Medicine, 2 (6) pp. 696-699.
Rescue of motoneurons from axotomy-induced cell death by polymer encapsulated cells genetically engineered to release CNTF.
Tan S.A., Déglon N., Zurn A.D., Baetge E.E., Bamber B., Kato A.C., Aebischer P., 1996. Cell Transplantation, 5 (5) pp. 577-587.
Encapsulation of neurotrophic factor-releasing cells for the treatment of neurodegenerative diseases.
Aebischer P., Tan S.A., Déglon N., Heyd B., Zurn A., Baetge E., Sagot Y., Kato A., 1995. Restorative Neurology and Neuroscience, 8 (1) pp. 65-66.
Fatty acids regulate Thy-1 antigen mRNA stability in T lymphocyte precursors.
Déglon N., Wilson A., Desponds C., Laurent P., Bron C., Fasel N., 1995. European Journal of Biochemistry, 231 (3) pp. 687-696.
Minimal membrane and secreted mu poly(A) signals specify developmentally-regulated immunoglobulin heavy chain mRNA ratios without RNA splicing.
Fasel N.J., Deglon N., Beghdadi-Rais C., Law R., Bron C., Wall R., 1994. Molecular Immunology, 31 (7) pp. 563-566.
Membrane mu poly(A) signal and 3' flanking sequences function as a transcription terminator for immunoglobulin-encoding genes.
Fasel N.J., Rousseaux M., Déglon N., Govan H.L., Law R., Bron C., Wall R., 1992. Gene, 122 (2) pp. 297-304.
Multi-level regulation of Thy-1 antigen expression in mouse T lymphomas.
Fasel N.J., Déglon N., 1992. Immunogenetics, 35 (2) pp. 126-130.
Evaluation of somatic cell variants deficient in glycosylphosphatidyl-inositol anchoring as candidates for genetic correction.
Fasel N., Déglon N., Rousseaux M., Beghdadi-Rais C., Bernard M., Bron C., 1991. Cell Biology International Reports, 15 (11) pp. 1051-1064.
Isolation from mouse fibroblasts of a cDNA encoding a new form of the fibroblast growth factor receptor (flg).
Fasel N.J., Bernard M., Déglon N., Rousseaux M., Eisenberg R.J., Bron C., Cohen G.H., 1991. Biochemical and Biophysical Research Communications, 178 (1) pp. 8-15.
Translocation of the yeast dolichol-phosphate-mannose synthase into microsomal membranes.
Déglon N., Krapp A., Bron C., Fasel N., 1991. Biochemical and Biophysical Research Communications, 174 (3) pp. 1337-1342.
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