M. F. Beal, E. Brouillet, B. G. Jenkins, R. J. Ferrante, N. W. Kowall et al., Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid, J. Neurosci, vol.13, pp.4181-4192, 1993.

N. Bizat, J. M. Hermel, F. Boyer, C. Jacquard, C. Creminon et al., Calpain is a major cell death effector in selective striatal degeneration induced in vivo by 3-nitropropionate: implications for Huntington's disease, J. Neurosci, vol.23, pp.5020-5030, 2003.

N. Bizat, J. M. Hermel, S. Humbert, C. Jacquard, C. Creminon et al., In Vivo Calpain/Caspase Cross-talk during 3-Nitropropionic Acid-induced Striatal Degeneration: IMPLICATION OF A CALPAIN-MEDIATED CLEAVAGE OF ACTIVE CASPASE-3, Journal of Biological Chemistry, vol.278, issue.44, pp.278-43245, 2003.
DOI : 10.1074/jbc.M305057200

D. Blum, D. Gall, L. Cuvelier, and S. N. Schiffmann, Topological analysis of striatal lesions induced by 3-nitropropionic acid in the Lewis rat, Neuroreport, vol.12, issue.8, pp.1769-1772, 2001.
DOI : 10.1097/00001756-200106130-00050

D. Blum, M. C. Galas, D. Gall, L. Cuvelier, and S. N. Schiffmann, Striatal and Cortical Neurochemical Changes Induced by Chronic Metabolic Compromise in the 3-Nitropropionic Model of Huntington's Disease, Neurobiology of Disease, vol.10, issue.3, pp.410-426, 2002.
DOI : 10.1006/nbdi.2002.0512

D. Blum, R. Hourez, M. C. Galas, P. Popoli, and S. N. Schiffmann, Adenosine receptors and Huntington's disease: implications for pathogenesis and therapeutics, The Lancet Neurology, vol.2, issue.6, pp.366-374, 2003.
DOI : 10.1016/S1474-4422(03)00411-3

D. Blum, M. C. Galas, A. Pintor, E. Brouillet, C. Ledent et al., A dual role of adenosine A2A receptors in 3-nitropropionic acid-induced striatal lesions: implications for the neuroprotective potential of A2A antagonists, J. Neurosci, vol.23, pp.5361-5369, 2003.

D. Blum, M. C. Galas, L. Cuvelier, and S. N. Schiffmann, Chronic intoxication with 3-nitropropionic acid in rats induces the loss of striatal dopamine terminals without affecting nigral cell viability, Neuroscience Letters, vol.354, issue.3, pp.354-234, 2004.
DOI : 10.1016/j.neulet.2003.10.034

E. Brouillet, F. Conde, M. F. Beal, and P. Hantraye, Replicating Huntington's disease phenotype in experimental animals, Progress in Neurobiology, vol.59, issue.5, pp.427-468, 1999.
DOI : 10.1016/S0301-0082(99)00005-2

C. Chatellard-causse, B. Blot, N. Cristina, S. Torch, M. Missotten et al., Alix (ALG-2-interacting Protein X), a Protein Involved in Apoptosis, Binds to Endophilins and Induces Cytoplasmic Vacuolization, Journal of Biological Chemistry, vol.277, issue.32, pp.277-29108, 2002.
DOI : 10.1074/jbc.M204019200

B. Chen, S. C. Borinstein, J. Gillis, V. W. Sykes, and O. Bogler, The Glioma-associated Protein SETA Interacts with AIP1/Alix and ALG-2 and Modulates Apoptosis in Astrocytes, Journal of Biological Chemistry, vol.275, issue.25, pp.275-19275, 2000.
DOI : 10.1074/jbc.M908994199

S. Fraboulet, F. J. Hemming, A. L. Mahul, N. Cristina, and R. Sadoul, Expression of Alix protein during early avian development, Gene Expression Patterns, vol.3, issue.2, pp.139-142, 2003.
DOI : 10.1016/S1567-133X(03)00025-5

J. Gafni and L. M. Ellerby, Calpain activation in Huntington's disease, J. Neurosci, vol.22, pp.4842-4849, 2002.

J. Gafni, E. Hermel, J. E. Young, C. L. Wellington, M. R. Hayden et al., Inhibition of Calpain Cleavage of Huntingtin Reduces Toxicity, Journal of Biological Chemistry, vol.279, issue.19, pp.279-20211, 2004.
DOI : 10.1074/jbc.M401267200

M. C. Galas, N. Bizat, L. Cuvelier, K. Bantubungi, E. Brouillet et al., Death of cortical and striatal neurons induced by mitochondrial defect involves differential molecular mechanisms, Neurobiology of Disease, vol.15, issue.1, pp.15-152, 2004.
DOI : 10.1016/j.nbd.2003.09.013

F. J. Hemming, S. Fraboulet, B. Blot, and R. Sadoul, Early increase of apoptosis-linked gene-2 interacting protein X in areas of kainate-induced neurodegeneration, Neuroscience, vol.123, issue.4, pp.887-895, 2004.
DOI : 10.1016/j.neuroscience.2003.10.036

K. Katoh, H. Shibata, H. Suzuki, A. Nara, K. Ishidoh et al., The ALG-2-interacting Protein Alix Associates with CHMP4b, a Human Homologue of Yeast Snf7 That Is Involved in Multivesicular Body Sorting, Journal of Biological Chemistry, vol.278, issue.40, pp.278-39104, 2003.
DOI : 10.1074/jbc.M301604200

K. B. Kegel, M. Kim, E. Sapp, C. Mcintyre, J. G. Castano et al., Huntingtin expression stimulates endosomal?lysosomal activity, endosome tubulation, and autophagy, J. Neurosci, vol.20, pp.7268-7278, 2000.

T. Kiechle, A. Dedeoglu, J. Kubilus, N. W. Kowall, M. F. Beal et al., Cytochrome c and caspase-9 expression in Huntington's disease, Neuromolecular. Med, vol.1, pp.183-195, 2002.

M. Maki, Y. Kitaura, H. Satoh, S. Ohkouchi, and H. Shibata, Structures , functions and molecular evolution of the penta-EF-hand Ca 2+ binding proteins, Biochim. Biophys. Acta, pp.1600-51, 2002.

M. Missotten, A. Nichols, K. Rieger, and R. Sadoul, Alix, a novel mouse protein undergoing calcium-dependent interaction with the apoptosis-linked-gene 2 (ALG-2) protein, Cell Death Differ, pp.124-129, 1999.

S. Ouary, N. Bizat, S. Altairac, H. Menetrat, V. Mittoux et al., Major strain differences in response to chronic systemic administration of the mitochondrial toxin 3-nitropropionic acid in rats: implications for neuroprotection studies, Neuroscience, vol.97, issue.3, pp.521-530, 2000.
DOI : 10.1016/S0306-4522(00)00020-8

A. V. Panov, C. A. Gutekunst, B. R. Leavitt, M. R. Hayden, J. R. Burke et al., Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines, Nature Neuroscience, vol.5, pp.731-736, 2002.
DOI : 10.1038/nn884

Z. H. Qin, Y. Wang, K. B. Kegel, A. Kazantsev, B. L. Apostol et al., Autophagy regulates the processing of amino terminal huntingtin fragments, Human Molecular Genetics, vol.12, issue.24, pp.12-3231, 2003.
DOI : 10.1093/hmg/ddg346

A. Sittler, S. Walter, N. Wedemeyer, R. Hasenbank, E. Scherzinger et al., SH3GL3 Associates with the Huntingtin Exon 1 Protein and Promotes the Formation of Polygln-Containing Protein Aggregates, Molecular Cell, vol.2, issue.4, pp.427-436, 1998.
DOI : 10.1016/S1097-2765(00)80142-2

B. Strack, A. Calistri, S. Craig, E. Popova, and H. G. Gottlinger, AIP1/ALIX Is a Binding Partner for HIV-1 p6 and EIAV p9 Functioning in Virus Budding, Cell, vol.114, issue.6, pp.689-699, 2003.
DOI : 10.1016/S0092-8674(03)00653-6

Y. Trioulier, S. Torch, B. Blot, N. Cristina, C. Chatellard-causse et al., Alix, a Protein Regulating Endosomal Trafficking, Is Involved in Neuronal Death, Journal of Biological Chemistry, vol.279, issue.3, pp.279-2046, 2004.
DOI : 10.1074/jbc.M309243200

URL : https://hal.archives-ouvertes.fr/inserm-00381892

P. Vito, E. Lacana, and L. D. Adamio, Interfering with Apoptosis: Ca2+-Binding Protein ALG-2 and Alzheimer's Disease Gene ALG-3, Science, vol.271, issue.5248, pp.521-525, 1996.
DOI : 10.1126/science.271.5248.521

P. Vito, L. Pellegrini, C. Guiet, and L. D. Adamio, Cloning of AIP1, a novel protein that associates with the apoptosis-linked gene ALG-2 in a Ca 2+ -dependent reaction, J. Biol. Chem, pp.274-1533, 1999.