Publications

Nóbrega, C., Nascimento-Ferreira, I., Onofre, I., Albuquerque, D.; Hirai, H.; Déglon, N., Pereira de Almeida, L. (2013). Silencing of mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic mice. PlosOne 8: e52396

Nascimento-Ferreira, I., Nobrega, C., Albuquerque, D., Déglon, N. and Pereira de Almeida, L. (2013). Overexpression of mutant ataxin-3 in mouse cerebellum induces ataxia and cerebellar neuropathology. Cerebellum (In Press)

Cambon K, and Déglon N. (2013). Lentiviral-mediated gene transfer of siRNAs for the treatment of Huntington' disease. Second edition of Trinucleotide Repeat Protocols book: From Mechanisms to Hope for Cure. Ed. Springer. (In Press)

Caillierez, R., Bégard S., Lécolle K., Zommer N., Loyens A., Dufour N., Aurégan G., Hantraye P., Déglon N., Buée L. and Colin M. (2013). Lentiviral delivery of human wild-type Tau mediates a slow and progressive neurodegenerative Tau pathology in the rat brain. Mol. Ther. (In Press)

Quesseveur G., David D.J., Gaillard M.C. Pla P., Wu M., Nguyen H.T., Nicolas V., Auregan G., David I., Dranowski A., Saudou F., Hantraye P., Hen R., Gardier A.M., Déglon N., Guiard B.P. (2013). BDNF overexpression in mouse hippocampal astrocytes promotes local neurogenesis and elicits anxiolytic-like activities. Translational Psychiatry (In Press)

Delzor, A., Dufour, N., and Déglon, N. (2013). Lentiviral vectors in Huntington’s disease research and therapy. Humana Press Book on “Viral Vectors in Neurobiology and Brain Diseases”. (In Press)

Nascimento-Ferreira, I., Nóbrega, C., Onofre, I., Albuquerque, D., Hirai, H.; Déglon, N., Pereira de Almeida, L. (2013). Beclin-1 protein mitigates motor and neuropathological deficits in lentiviral and transgenic mouse models of Machado-Joseph disease. Brain (In Press)

Delzor, A., Dufour, N., Petit, F., Guillermier, M., Houitte, D., Auregan G., Brouillet, E., Hantraye, P., and Déglon, N. (2012). Restricted transgene expression in the brain with cell-type specific neuronal promoters. Hum. Gene Ther Meth. 23: 242-254

Ruiz, M., and Déglon, N (2012). Viral-mediated overexpression of mutant huntingtin to model HD in various species. Neurobiol. Dis. 48: 202-212

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

Simões, A.T., Gonçalves, N., Déglon, N., Kügler, S., Bandeira Duarte, C., Pereira de Almeida, L. (2012). Calpastatin-mediated inhibition of calpains in the mouse brain prevents mutant ataxin-3 proteolysis, translocation to the nucleus and aggregation, relieving Machado-Joseph disease. Brain. 135: 2428-2439

Galvan, L., Lepejová, N., Gaillard, M.-C., Malgorn, C., Guillermier, M., Houitte, D., Bonvento, G., Petit, F., Dufour, N., Héry, P., Gérard, M., Elalouf, J.-M., Déglon, N., Brouillet, E., de Chaldée, M. (2012). The newly identified striatal protein Capucin (Tmem90a) modulates toxicity of N-terminal fragment of mutant Htt in vivo. Neurobiol. Aging 33: 1845.e5–1845.e6

Colin, A, Faideau, M., Dufour, N., Auregan, G., Hassig, R., Escartin, C., Hantraye, P., Bonvento, G. and Déglon N. (2012). MicroRNA’s detargeting technology in the context of CNS applications. The Clinibook: Clinical gene transfer. Ed. EDK, Paris. pp.129-133

Nascimento-Ferreira, I., Santos-Ferreira, T., Sousa-Ferreira, L., Auregan, G., Alves, S., Dufour, N., Colomer, V., Koeppen, A, Déglon, N. and Pereira de Almeida, L. (2011). Degradation pathways in Machado-Joseph Disease: Recovery of mutant ataxin-3 clearance through overexpression of the autophagy-related beclin-1 protein. Brain 134: 1400-1415

Martin, E., Betuing, S., Pagès, C., Cambon, K., Auregan, G., Déglon, N., Caboche, J., Roze, E. (2011). Mitogen and Stress-activated protein Kinase 1 and neuroprotection in Huntington’s Disease: a role on chromatin remodeling at the promoter of PGC-1alpha. Hum. Mol. Genet. 20: 2422-2434

Escartin, C., Won, S.J., Chen, P.-C., Auregan, G., Déglon, N., Berman, A.E., Johnson, J.A., Suh, S.W., Swanson, R.A. (2011). Nrf2 facilitates neuronal glutathione synthesis by up-regulating neuronal EAAT3 expression. J. Neurosci. 31: 7392-7401

Damiano, M. Galvan, L., Déglon, N. and Brouillet, E. (2010). Mitochondria in Huntington’s disease. Biochem. Biophys. Acta 1802: 52-61

Rudinskiy, N., Kaneko, Y.A., Beesen, A.A., Gokce, O., Régulier, R., Déglon, N. and Luthi-Carter, R. (2009). Diminished hippocalcin expression in Huntington’s disease (HD) brain does not account for increased striatal neuron vulnerability as assessed in primary neuron models of HD. J. Neurochem. 111: 460-72

Alves, S., Nascimento-Ferreira, I., Dufour, N., Hassig, R., Auregan, G., Brouillet, E., Hantraye, P., Déglon, N., and Pereira de Almeida, L. (2010). Silencing ataxin-3 mitigates degeneration in a rat model of Machado-Joseph disease: no role for wild-type ataxin-3? Hum. Mol. Genet. 19:2380-94

Faideau, M., Kim J., Cormier, K., Glimore, R., Welch, M., Auregan, G., Dufour, N., Guillermier, M., Brouillet, E., Hantraye, P., Déglon, N., Ferrante, R.J., Bonvento, G. (2010). In vivo expression of polyglutamine-expanded huntingtin by mouse striatal astrocytes impairs glutamate transport: a correlation with Huntington's disease subjects. Hum. Mol. Genet. 19: 3053–3067
 

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

Perrin, V., Dufour, N., Raoul, C., T, Aebischer, P., Luthi-Carter, R., and Déglon N. (2009). Activation of JNK pathway in a rat model of HD and implication in the pathology. Exp. Neurol. 215: 191-200

Lundberg, C., Björklund, T., Carlsson, T., Jakobsson, J., Hantraye, P., Déglon, N. and Kirik, D. (2008). Applications of lentiviral vectors for biology and gene therapy of neurological disorders. Curr. Gene Ther. 8: 461-473

Leriche, L., Björklund, T., Breysse, N., Besret, L., Grégoire, M.-C., Carlsson, T., Dollé, F., Mandel, RJ, Déglon, N., Hantraye, P. and Kirik, D. (2009). PET imaging demonstrates correlation between behavioral recovery and correction of dopamine neurotransmission following gene therapy. J. Neurosci. 29: 1544-1553

Diguet, E., Petit, F., Escartin, C., Cambon, K., Bizat, N., Dufour, N., Hantraye, P., Déglon, N., and Brouillet E. (2009). Normal brain aging modulates the toxicity of mutant huntingtin. PLoS ONE: 4(2):e4637

Ciron C, Cressant A, Roux F, Raoul S, Cherel Y, Hantraye P, Déglon N, Schwartz B, Barkats M, Heard JM, Tardieu M, Moullier P, Colle MA. (2009). Human alpha-Iduronidase Gene Human α-Iduronidase Gene Transfer Mediated by Adeno-Associated Virus Types 1, 2, and 5 in the Brain of Nonhuman Primates: Vector Diffusion and Biodistribution. Hum. Gene Ther. 20: 350-360

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
 

Charvin, D., Roze, E, Perrin, V., Deyts, C., Betuing, S, Pagès, C., Régulier, E., Luthi-Carter, R., Brouillet, E., Déglon, N., and Caboche J. (2008). Haloperidol protects striatal neurons from dysfunction induced by mutated Huntingtin in vivo. Neurobiol. Dis. 29: 22-29

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

Alves, S., Régulier, E., Ferreira, I, Hassig, R., Dufour, N., Koeppen, A., Carvalho, A.L., Simões, S., Pedroso de Lima C., Brouillet, E., Colomer-Gould, V.F., Déglon N., Pereira de Almeida, L. (2008). Striatal and nigral pathology in a lentiviral rat model of Machado-Joseph disease. Hum. Mol. Genet. 17: 2071-2083.

Prost S, Le Dantec M, Augé S, Le Grand R, Derdouch S, Auregan G, Déglon N, Relouzat F, Aubertin AM, Maillere B, Dusanter-Fourt I, Kirszenbaum M. (2008). Human and simian immunodeficiency viruses deregulate early hematopoiesis through a Nef/PPARgamma/STAT5 signaling pathway in macaques. J. Clin. Invest. 118: 1765-11775.

Prost S, Le Dantec M, Augé S, Le Grand R, Derdouch S, Auregan G, Déglon N, Relouzat F, Aubertin AM, Maillere B, Dusanter-Fourt I, Kirszenbaum M. (2008). Nef and PPAR-gamma interact to suppress Stat5 expression in CD34+ progenitors from infected macaques. Med Sci (Paris) 24:551-554.

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

Alves, S., Nascimento-Ferreira, I., Auregan, G., Hassig, R., Dufour, N., Brouillet, E., Pedroso de Lima, M.C., Hantraye, P., Pereira de Almeida, L., and Déglon, N. (2008). Allele-specific RNA silencing of mutant ataxin-3 mediates neuroprotection in a rat model of Machado-Joseph disease. PlosOne 3 (10): e3341
 

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

Bloch, J., Bachoud-Lévi, A.C., Déglon, N., Lefaucheur, J.P., Winkel, L., Nguyen, J.P., Bourdet, C., Rémy, P., Brugières, P., Boissé, M-F., Baudic, S., Césaro, P., Hantraye, P., Aebischer, P. and Peschanski, M. (2004). Neuroprotective gene therapy for Huntington’s disease using polymer encapsulated cells engineered to secrete human CNTF: results of a phase I study. Hum. Gene Ther. 15 : 968-975

Abbas-Terki, T., Blanco-Bose, W., Déglon, N., Pralong, W., Aebischer, P. (2002). Lentiviral-mediated RNA interference. Hum. Gene Ther. 13: 2197-2201

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

Bachoud-Lévi, A.-C., Déglon N., Nguyen, J.-P., Bloch, J., Bourdet, C., Rémy, P., Winkel, L., Lefaucheur, J.-P., Goddard, M., Brugières, P., Baudic, S., Cesaro, P., Peschanski, M. and Aebischer, P. (2000). Neuroprotective gene therapy for Huntington’s disease using a polymer encapsulated BHK cell line engineered to secreted human CNTF. Hum. Gene Ther. 11, 1723-1729

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

Kordower, J.H., Bloch, J., Ma, S., Chu, Y., Palfi, S., Roitberg, B., Emborg, M., Hantraye, P., Déglon, N. and Aebischer, P. (1999). Lentiviral gene transfer to the nonhuman primate brain. Exp. Neurol. 160, 1-16

Déglon, N., Tan, S.A., Heyd, B., Joseph, J.-M., Zurn, A.D., and Aebischer, P. (1996). Central nervous system delivery of recombinant ciliary neurotrophic factor by polymer encapsuled differentiated C2C12 myoblasts. Hum. Gene Ther. 7, 2135-2146

Aebischer, P., Schluep, M., Déglon, N., Joseph, J.-M., Hirt, L., Heyd, B., Goddard, M., Hammang, J., Zurn, A., Kato, A., Regli, F., and Baetge, E.E. (1996). Intrathecal delivery of CNTF using encapsulated genetically modified xenogeneic cells in amyotrophic lateral sclerosis patients. Nature Med. 2, 696-699

Aebischer P., Pochon, N.A.-M., Heyd, B., Déglon, N., Joseph, J.-M., Zurn, A., Baetge, E.E., Hammang, J.P., Goddard, M., Lysaght, M., Kaplan, F., Kato, A.C., Schluep, M., Hirt, L., Regli, F., Porchet, F., and de Tribolet, N. (1996). Gene therapy for amyotrophic lateral sclerosis (ALS) using a polymer encapsulated xenogenic cell line engineered to secrete hCNTF: Clinical protocol. Hum. Gene Ther. 7, 851-860