2014 | 2013 | 2012 | 2011 | 2010 | Selected Publications


Nóbrega, C., Nascimento-Ferreira, I., Onofre, I., Albuquerque, D., Déglon, N., Pereira de Almeida, L. (2014). RNA interference mitigates motor and neuropathological deficits in a cerebellar mouse model of Machado-Joseph disease. PlosOne 9(8): e100086. doi:10.1371/journal.pone.0100086

Drouet, V.,Ruiz, M., Zala, D., Feyeux M., Auregan, G., Cambon, K., Troquier, L., Carpentier, J., Aubert, S., Merienne, N., Bourgois-Rocha, F., Hassig, R., Rey, M., Dufour, N., Saudou F., Perrier A., Hantraye, P., and Déglon, N. (2014). Allele-specific silencing of mutant huntingin in rodent brain and human stem cells. PlosOne 9(6): e99341. doi:10.1371/journal.pone.0099341

Facchinetti, P., Dorard, E., Contremoulins, V., Gaillard, M.-C., Déglon, N., Sazdovitch, V., Guihenneuc-Jouyaux, C., Brouillet, E., Duyckaerts, C., Allinquant, B. (2014). SET translocation is associated with increase in caspase cleaved amyloid precursor protein, in CA1 of Alzheimer and Down syndrome patients. Neurobiol. Aging 35: 958-968

Dujardin, S., Lécolle, K., Caillierez, R., Bégard, S., Zommer, N., Lachaud, C., Carrier, S., Dufour, N., Aurégan, N., Winderickx, J., Hantraye, P., Déglon, N., Colin, M., and Buée, L. Neuron-to-neuron wild-type Tau protein transfer through a trans-synaptic mechanism: Relevance to sporadic tauopathies (2014). Acta Neuropathol. Comm. 2: 14. doi: 10.1186/2051-5960-2-14

Pannasch, U., Freche, D., Knott, G., Escartin, C., Ezan, P., Cohen-Salmon, M., Benchenane, K., Abudara, V., Déglon, N., Giaume, C., Holcman, D., Rouach, N (2014). Connexin 30 controls synaptic strength through astroglial synapse invasion. Nat. Neurosci. 17: 549-558

Delzor, A., Dufour, N., and Déglon, N. (2014). Lentiviral vectors in Huntington’s disease research and therapy. Humana Press Book on “Viral Vectors in Neurobiology and Brain Diseases”. Volume 82, pp. 193-220



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

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. 21: 1358-1368

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. Transl Psychiatry 3: e253, doi: 10.1038/tp.2013.30

Cambon K, and Déglon N. (2013). Lentiviral-mediated gene transfer of siRNAs for the treatment of Huntington' disease. Methods Mol. Biol. 1010: 95-109.

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 136: 2173-2188

Mérienne, N., Le Douce, J., Faivre, E., Déglon N. and Bonvento, G. (2013). Efficient gene delivery and selective transduction of astrocytes in the mammalian brain using viral vectors. Front. Cell. Neurosci. 7: e106, DOI: 10.3389/fncel.2013.00106

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 12: 441-455

Damiano, M., Diguet, E., Malgorn, C., Galvan, L., Petit, F., Benhaim, L., Guillermier, M., Houitte, D., Dufour, N., Hantraye, P., Canals, J., Alberch, J., Delzescaux, T., Déglon, N., Beal, M.F., Brouillet, E. (2013). Mitochondrial complex II defects in genetic models of Huntington's disease expressing N-terminal fragments of mutant huntingtin. Hum. Mol. Genet. 22: 3869-3882

Delzor, A., Escartin, C., and Déglon N. (2013). Lentiviral vectors: a powerful tool to target astrocytes in vivo. Current Drug Targets 14: 1336-1346






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


Selected Publications

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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