S. Prusiner and . Prions, Nobel Lecture: Prions, Proceedings of the National Academy of Sciences, vol.389, issue.6650, pp.13363-83, 1998.
DOI : 10.1038/39097

C. Cunningham, R. Deacon, K. Chan, D. Boche, J. Rawlins et al., Neuropathologically distinct prion strains give rise to similar temporal profiles of behavioral deficits, Neurobiology of Disease, vol.18, issue.2, pp.258-69, 2005.
DOI : 10.1016/j.nbd.2004.08.015

R. Deacon, J. Raley, V. Perry, and J. Rawlins, Burrowing into prion disease, Neuroreport, vol.12, issue.9, pp.2053-2060, 2001.
DOI : 10.1097/00001756-200107030-00052

K. Guenther, R. Deacon, V. Perry, and J. Rawlins, Early behavioural changes in scrapie-affected mice and the influence of dapsone, European Journal of Neuroscience, vol.123, issue.2, pp.401-410, 2001.
DOI : 10.1002/(sici)1520-6777(1998)17:5<555::aid-nau12>3.0.co;2-e

C. Cunningham, R. Deacon, H. Wells, D. Boche, S. Waters et al., Synaptic changes characterize early behavioural signs in the ME7 model of murine prion disease, European Journal of Neuroscience, vol.144, issue.10, pp.2147-55, 2003.
DOI : 10.1046/j.1460-9568.2003.02662.x

R. Chiesa, P. Piccardo, S. Dossena, L. Nowoslawski, K. Roth et al., Bax deletion prevents neuronal loss but not neurological symptoms in a transgenic model of inherited prion disease, Proceedings of the National Academy of Sciences, vol.30, issue.4, pp.238-281, 2005.
DOI : 10.1002/ana.410300410

E. Jamieson, M. Jeffrey, J. Ironside, and J. Fraser, Apoptosis and dendritic dysfunction precede prion protein accumulation in 87V scrapie, Neuroreport, vol.12, issue.10, pp.2147-53, 2001.
DOI : 10.1097/00001756-200107200-00021

G. Mallucci, Prion neurodegeneration, Prion, vol.106, issue.4, pp.195-201, 2009.
DOI : 10.1038/nature07761

Z. Siskov-a, D. Mahad, C. Pudney, G. Campbell, M. Cadogan et al., Morphological and Functional Abnormalities in Mitochondria Associated with Synaptic Degeneration in Prion Disease, The American Journal of Pathology, vol.177, issue.3
DOI : 10.2353/ajpath.2010.091037

Z. Li, K. Okamoto, Y. Hayashi, and M. Sheng, The Importance of Dendritic Mitochondria in the Morphogenesis and Plasticity of Spines and Synapses, Cell, vol.119, issue.6, pp.873-87, 2004.
DOI : 10.1016/j.cell.2004.11.003

P. Trimmer, R. Swerdlow, J. Parks, P. Keeney, J. Bennett et al., Abnormal Mitochondrial Morphology in Sporadic Parkinson's and Alzheimer's Disease Cybrid Cell Lines, Experimental Neurology, vol.162, issue.1, pp.37-507333, 2000.
DOI : 10.1006/exnr.2000.7333

R. Castellani, K. Hirai, G. Aliev, K. Drew, A. Nunomura et al., Role of mitochondrial dysfunction in Alzheimer's disease, Journal of Neuroscience Research, vol.44, issue.3, pp.357-60, 2002.
DOI : 10.1002/jnr.10389

T. Dawson and V. Dawson, Molecular Pathways of Neurodegeneration in Parkinson's Disease, Science, vol.302, issue.5646, pp.819-841, 2003.
DOI : 10.1126/science.1087753

M. Beal, Mitochondria take center stage in aging and neurodegeneration, Annals of Neurology, vol.38, issue.4, pp.495-505, 2005.
DOI : 10.1002/ana.20624

C. Grenier, C. Bissonnette, L. Volkov, and X. Roucou, Molecular morphology and toxicity of cytoplasmic prion protein aggregates in neuronal and non-neuronal cells, Journal of Neurochemistry, vol.280, issue.5, pp.1456-66, 2006.
DOI : 10.1073/pnas.97.1.145

V. Mattei, P. Matarrese, T. Garofalo, A. Tinari, L. Gambardella et al., Recruitment of cellular prion protein to mitochondrial raft-like microdomains contributes to apoptosis execution, Molecular Biology of the Cell, vol.22, issue.24, pp.4842-53, 2011.
DOI : 10.1091/mbc.E11-04-0348

N. Hachiya, M. Yamada, K. Watanabe, A. Jozuka, T. Ohkubo et al., Mitochondrial localization of cellular prion protein (PrPC) invokes neuronal apoptosis in aged transgenic mice overexpressing PrPC, Neuroscience Letters, vol.374, issue.2, pp.98-103, 2005.
DOI : 10.1016/j.neulet.2004.10.044

X. Wang, C. Dong, Q. Shi, S. Shi, G. Wang et al., Cytosolic prion protein induces apoptosis in human neuronal cell SH-SY5Y via mitochondrial disruption pathway, BMB Reports, vol.42, issue.7, pp.444-453, 2009.
DOI : 10.5483/BMBRep.2009.42.7.444

J. Carimalo, S. Cronier, G. Petit, J. Peyrin, F. Boukhtouche et al., Activation of the JNK-c-Jun pathway during the early phase of neuronal apoptosis induced by PrP106-126 and prion infection, European Journal of Neuroscience, vol.389, issue.9, pp.2311-2320, 2005.
DOI : 10.1111/j.1460-9568.2005.04080.x

URL : https://hal.archives-ouvertes.fr/hal-00081975

O. Donovan, C. Tobin, D. Cotter, and T. , Prion Protein Fragment PrP-(106-126) Induces Apoptosis via Mitochondrial Disruption in Human Neuronal SH-SY5Y Cells, Journal of Biological Chemistry, vol.276, issue.47, pp.43516-43539, 2001.
DOI : 10.1074/jbc.M103894200

I. Dupiereux, W. Zorzi, W. Rachidi, D. Zorzi, O. Pierard et al., Study on the toxic mechanism of prion protein peptide 106???126 in neuronal and non neuronal cells, Journal of Neuroscience Research, vol.20, issue.3, pp.637-683, 2006.
DOI : 10.1002/jnr.20965

C. Crozet, F. Beranger, and S. Lehmann, Cellular pathogenesis in prion diseases 39:44; PMID:18413130, Vet Res, vol.http, 2008.
DOI : 10.1051/vetres:2008021

N. Stahl, D. Borchelt, K. Hsiao, and S. Prusiner, Scrapie prion protein contains a phosphatidylinositol glycolipid PMID:2444340, Cell, vol.5187, pp.229-400092, 1987.

P. Orlean and A. Menon, Thematic review series: Lipid Posttranslational Modifications. GPI anchoring of protein in yeast and mammalian cells, or: how we learned to stop worrying and love glycophospholipids, The Journal of Lipid Research, vol.48, issue.5, pp.993-1011, 2007.
DOI : 10.1194/jlr.R700002-JLR200

K. Ohishi, N. Inoue, T. Kinoshita, and P. Pig-s, PIG-S and PIG-T, essential for GPI anchor attachment to proteins, form a complex with GAA1 and GPI8, The EMBO Journal, vol.20, issue.15, pp.4088-98, 2001.
DOI : 10.1093/emboj/20.15.4088

I. Caras, Probing the signal for glycophosphatidylinositol anchor attachment using decay accelerating factor as a model system, Cell Biology International Reports, vol.15, issue.9, pp.815-260309, 1991.
DOI : 10.1016/0309-1651(91)90035-H

M. Hoque, T. Kitamoto, H. Furukawa, T. Muramoto, and J. Tateishi, Mutation in the prion protein gene at codon 232 in Japanese patients with Creutzfeldt-Jakob disease: a clinicopathological, immunohistochemical and transmission study, Acta Neuropathologica, vol.92, issue.5, pp.441-447, 1996.
DOI : 10.1007/s004010050544

O. Windl, A. Giese, W. Schulz-schaeffer, I. Zerr, K. Skworc et al., Molecular genetics of human prion diseases in Germany, Human Genetics, vol.105, issue.3, pp.244-52, 1999.
DOI : 10.1007/s004390051096

G. Guizzunti and C. Zurzolo, The Fate of PrP GPI-Anchor Signal Peptide is Modulated by P238S Pathogenic Mutation, Traffic, vol.27, issue.Pt 10, pp.78-93, 2014.
DOI : 10.1111/tra.12126

URL : https://hal.archives-ouvertes.fr/pasteur-00874727

T. Sommer and S. Jentsch, A protein translocation defect linked to ubiquitin conjugation at the endoplasmic reticulum, Nature, vol.365, issue.6442, pp.176-185, 1993.
DOI : 10.1038/365176a0

C. Rodighiero, B. Tsai, T. Rapoport, and W. Lencer, Role of ubiquitination in retro-translocation of cholera toxin and escape of cytosolic degradation, EMBO Reports, vol.3, issue.12, pp.1222-1229, 2002.
DOI : 10.1093/embo-reports/kvf239

M. De-virgilio, H. Weninger, and N. Ivessa, Ubiquitination Is Required for the Retro-translocation of a Short-lived Luminal Endoplasmic Reticulum Glycoprotein to the Cytosol for Degradation by the Proteasome, Journal of Biological Chemistry, vol.273, issue.16, pp.9734-9777, 1998.
DOI : 10.1074/jbc.273.16.9734

Y. Gu, A. Singh, S. Bose, and N. Singh, Pathogenic mutations in the glycosylphosphatidylinositol signal peptide of PrP modulate its topology in neuroblastoma cells, Molecular and Cellular Neuroscience, vol.37, issue.4, pp.647-56, 2008.
DOI : 10.1016/j.mcn.2007.08.018

S. Susin, H. Lorenzo, N. Zamzami, I. Marzo, B. Snow et al., Molecular characterization of mitochondrial apoptosis-inducing factor, Nature, vol.397, pp.441-447, 1999.

R. Haugland, Molecular Probes: Handbook of Fluorescent Probes and Research Chemicals, pp.266-267, 1996.

M. Poot, Y. Zhang, J. Kramer, K. Wells, L. Jones et al., Analysis of mitochondrial morphology and function with novel fixable fluorescent stains., Journal of Histochemistry & Cytochemistry, vol.44, issue.12, pp.1363-72, 1996.
DOI : 10.1177/44.12.8985128

V. Johnson, S. Ko, T. Holmstrom, J. Eriksson, and S. Chow, Effector caspases are dispensable for the early nuclear morphological changes during chemicalinduced apoptosis, J Cell Sci, vol.113, pp.2941-5310934034, 2000.