F. Ahmad and P. Baas, Microtubules released from the neuronal centrosome are transported into the axon, J Cell Sci, vol.108, pp.2761-2769, 1995.

A. Bhamidipati, S. Lewis, and N. Cowan, Adp Ribosylation Factor-like Protein 2 (Arl2) Regulates the Interaction of Tubulin-Folding Cofactor D with Native Tubulin, The Journal of Cell Biology, vol.124, issue.5, pp.1087-1096, 2000.
DOI : 10.1016/S0092-8674(00)81446-4

S. Boillee, V. Velde, C. Cleveland, and D. , ALS: A Disease of Motor Neurons and Their Nonneuronal Neighbors, Neuron, vol.52, issue.1, pp.39-59, 2006.
DOI : 10.1016/j.neuron.2006.09.018

H. Bömmel, G. Xie, W. Rossoll, S. Wiese, S. Jablonka et al., , a model of human motoneuron disease, The Journal of Cell Biology, vol.18, issue.4, pp.563-569, 2002.
DOI : 10.1016/S0092-8674(01)00363-4

T. Bordet, H. Schmalbruch, B. Pettmann, A. Hagège, L. Castelnau-ptakhine et al., Adenoviral cardiotrophin-1 gene transfer protects pmn mice from progressive motor neuronopathy, Journal of Clinical Investigation, vol.104, issue.8, pp.1077-1085, 1999.
DOI : 10.1172/JCI6265

URL : http://www.ncbi.nlm.nih.gov/pmc/articles/PMC408570

W. Bradley, L. Lassman, G. Pearce, and J. Walton, The neuromyopathy of vincristine in man, Journal of the Neurological Sciences, vol.10, issue.2, pp.107-131, 1970.
DOI : 10.1016/0022-510X(70)90013-4

E. Brandon, T. Szul, C. Alvarez, R. Grabski, R. Benjamin et al., On and Off Membrane Dynamics of the Endoplasmic Reticulum-Golgi Tethering Factor p115 In Vivo, Molecular Biology of the Cell, vol.17, issue.7, pp.2996-3008, 2006.
DOI : 10.1091/mbc.E05-09-0862

A. Brunialti, C. Poirier, H. Schmalbruch, and J. Guenet, The Mouse Mutation Progressive Motor Neuronopathy (pmn) Maps to Chromosome 13, Genomics, vol.29, issue.1, pp.131-135, 1995.
DOI : 10.1006/geno.1995.1223

B. Campenot, K. Lund, and D. Senger, Delivery of newly synthesized tubulin to rapidly growing distal axons of sympathetic neurons in compartmented cultures, The Journal of Cell Biology, vol.135, issue.3, pp.701-709, 1996.
DOI : 10.1083/jcb.135.3.701

R. Campenot, J. Soin, M. Blacker, K. Lund, H. Eng et al., Block of slow axonal transport and axonal growth by brefeldin A in compartmented cultures of rat sympathetic neurons, Neuropharmacology, vol.44, issue.8, pp.1107-1117, 2003.
DOI : 10.1016/S0028-3908(03)00042-X

N. Cashman, H. Durham, J. Blusztajn, K. Oda, T. Tabira et al., Neuroblastoma ?? spinal cord (NSC) hybrid cell lines resemble developing motor neurons, Developmental Dynamics, vol.319, issue.3, pp.209-221, 1992.
DOI : 10.1002/aja.1001940306

J. Cavanagh, The significance of the " dying back " process in experimental and human neurological disease, Int Rev Exp Pathol, vol.3, pp.219-267, 1964.

C. Cifuentes-diaz, N. S. Velasco, M. Borra-cebrian, C. Panozzo, C. Frugier et al., Neurofilament accumulation at the motor endplate and lack of axonal sprouting in a spinal muscular atrophy mouse model, Human Molecular Genetics, vol.11, issue.12, pp.1439-1447, 2002.
DOI : 10.1093/hmg/11.12.1439

M. Coleman, Axon degeneration mechanisms: commonality amid diversity, Nature Reviews Neuroscience, vol.62, issue.11, pp.889-898, 2005.
DOI : 10.1016/S0014-2999(99)00280-0

L. Conforti, R. Adalbert, and M. Coleman, Neuronal death: where does the end begin?, Trends in Neurosciences, vol.30, issue.4, pp.159-166, 2007.
DOI : 10.1016/j.tins.2007.02.004

V. Cullen, J. Brownlees, S. Banner, B. Anderton, P. Leigh et al., Gigaxonin is associated with the Golgi and dimerises via its BTB domain, NeuroReport, vol.15, issue.5, pp.873-876, 2004.
DOI : 10.1097/00001756-200404090-00028

R. Devon, P. Orban, K. Gerrow, M. Barbieri, C. Schwab et al., Als2-deficient mice exhibit disturbances in endosome trafficking associated with motor behavioral abnormalities, Proceedings of the National Academy of Sciences, vol.103, issue.25, pp.9595-9600, 2006.
DOI : 10.1073/pnas.0510197103

J. Donaldson, D. Finazzi, and R. Klausner, Brefeldin A inhibits Golgi membrane-catalysed exchange of guanine nucleotide onto ARF protein, Nature, vol.360, issue.6402, pp.350-352, 1992.
DOI : 10.1038/360350a0

H. Eng, K. Lund, and R. Campenot, Synthesis of -tubulin, actin, and other proteins in axons of sympathetic neurons in compartmented cultures, J Neurosci, vol.19, pp.1-9, 1999.

G. Feng, R. Mellor, M. Bernstein, C. Keller-peck, Q. Nguyen et al., Imaging Neuronal Subsets in Transgenic Mice Expressing Multiple Spectral Variants of GFP, Neuron, vol.28, issue.1, pp.41-51, 2000.
DOI : 10.1016/S0896-6273(00)00084-2

A. Ferri, J. Sanes, M. Coleman, J. Cunningham, and A. Kato, Inhibiting Axon Degeneration and Synapse Loss Attenuates Apoptosis and Disease Progression in a Mouse Model of Motoneuron Disease, Current Biology, vol.13, issue.8, pp.669-673, 2003.
DOI : 10.1016/S0960-9822(03)00206-9

L. Fischer, D. Culver, P. Tennant, A. Davis, M. Wang et al., Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man, Experimental Neurology, vol.185, issue.2, pp.232-240, 2004.
DOI : 10.1016/j.expneurol.2003.10.004

S. Forss-petter, P. Danielson, S. Catsicas, E. Battenberg, J. Price et al., Transgenic mice expressing ??-galactosidase in mature neurons under neuron-specific enolase promoter control, Neuron, vol.5, issue.2, pp.187-197, 1990.
DOI : 10.1016/0896-6273(90)90308-3

D. Frey, C. Schneider, L. Xu, J. Borg, W. Spooren et al., Early and selective loss of neuromuscular synapse subtypes with low sprouting competence in motoneuron diseases, J Neurosci, vol.20, pp.2534-2542, 2000.

J. Griffin and D. Watson, Axonal transport in neurological disease, Annals of Neurology, vol.270, issue.1, pp.3-13, 1988.
DOI : 10.1002/ana.410230103

G. Haase, P. Kennel, B. Pettmann, E. Vigne, S. Akli et al., Gene therapy of murine motor neuron disease using adenoviral vectors for neurotrophic factors, Nature Medicine, vol.17, issue.4, pp.429-436, 1997.
DOI : 10.1016/0165-3806(86)90208-7

M. Hafezparast, R. Klocke, C. Ruhrberg, A. Marquardt, A. Ahmad-annuar et al., Mutations in Dynein Link Motor Neuron Degeneration to Defects in Retrograde Transport, Science, vol.300, issue.5620, pp.808-812, 2003.
DOI : 10.1126/science.1083129

P. Hoffman, M. Lopata, D. Watson, and R. Luduena, Axonal transport of class II and III beta-tubulin: evidence that the slow component wave represents the movement of only a small fraction of the tubulin in mature motor axons, The Journal of Cell Biology, vol.119, issue.3, pp.595-604, 1992.
DOI : 10.1083/jcb.119.3.595

C. Howe and W. Mobley, Long-distance retrograde neurotrophic signaling, Current Opinion in Neurobiology, vol.15, issue.1, pp.40-48, 2005.
DOI : 10.1016/j.conb.2005.01.010

C. Infante, F. Ramos-morales, C. Fedriani, M. Bornens, and R. Rios, GMAP-210, A Cis-Golgi Network-associated Protein, Is a Minus End Microtubule-binding Protein, The Journal of Cell Biology, vol.266, issue.1, pp.83-98, 1999.
DOI : 10.1083/jcb.136.1.71

A. Jacquier, E. Buhler, M. Schafer, D. Bohl, S. Blanchard et al., Alsin/Rac1 signaling controls survival and growth of spinal motoneurons, Annals of Neurology, vol.302, issue.pt 2, pp.105-117, 2006.
DOI : 10.1002/ana.20886

URL : https://hal.archives-ouvertes.fr/hal-00089726

P. Kennel, F. Finiels, F. Revah, and J. Mallet, Neuromuscular function impairment is not caused by motor neurone loss in FALS mice, NeuroReport, vol.7, issue.8, pp.1427-1431, 1996.
DOI : 10.1097/00001756-199605310-00021

P. Kennel, P. Fonteneau, E. Martin, J. Schmidt, M. Azzouz et al., Electromyographical and Motor Performance Studies in thepmnMouse Model of Neurodegenerative Disease, Neurobiology of Disease, vol.3, issue.2, pp.137-147, 1996.
DOI : 10.1006/nbdi.1996.0014

T. Kimura, H. Watanabe, A. Iwamatsu, and K. Kaibuchi, Tubulin and CRMP-2 complex is transported via Kinesin-1, Journal of Neurochemistry, vol.161, issue.6, pp.1371-1382, 2005.
DOI : 10.1111/j.1471-4159.2005.03063.x

R. Klausner, J. Donaldson, and J. Lippincott-schwartz, Brefeldin A: insights into the control of membrane traffic and organelle structure, The Journal of Cell Biology, vol.116, issue.5, pp.1071-1080, 1992.
DOI : 10.1083/jcb.116.5.1071

B. Lamonte, K. Wallace, B. Holloway, S. Shelly, J. Ascano et al., Disruption of Dynein/Dynactin Inhibits Axonal Transport in Motor Neurons Causing Late-Onset Progressive Degeneration, Neuron, vol.34, issue.5, pp.715-727, 2002.
DOI : 10.1016/S0896-6273(02)00696-7

L. Langford and R. Schmidt, An electron microscopic analysis of the left phrenic nerve in the rat, The Anatomical Record, vol.25, issue.2, pp.207-213, 1983.
DOI : 10.1002/ar.1092050211

M. Larocca, J. M. Goldenring, and J. , AKAP350 modulates microtubule dynamics, European Journal of Cell Biology, vol.85, issue.7, pp.611-619, 2006.
DOI : 10.1016/j.ejcb.2005.10.008

J. Lippincott-schwartz, J. Donaldson, A. Schweizer, E. Berger, H. Hauri et al., Microtubule-dependent retrograde transport of proteins into the ER in the presence of brefeldin a suggests an ER recycling pathway, Cell, vol.60, issue.5, pp.821-836, 1990.
DOI : 10.1016/0092-8674(90)90096-W

N. Martin, J. Jaubert, P. Gounon, E. Salido, G. Haase et al., A missense mutation in Tbce causes progressive motor neuronopathy in mice, Nature Genetics, vol.666, issue.3, pp.443-447, 2002.
DOI : 10.1038/ng1016

Y. Mimori-kiyosue, I. Grigoriev, G. Lansbergen, H. Sasaki, C. Matsui et al., CLASP1 and CLASP2 bind to EB1 and regulate microtubule plus-end dynamics at the cell cortex, The Journal of Cell Biology, vol.112, issue.1, pp.141-153, 2005.
DOI : 10.1016/S0960-9822(01)00002-1

H. Mitsumoto and W. Bradley, MURINE MOTOR NEURON DISEASE (THE WOBBLER MOUSE), Brain, vol.105, issue.4, pp.811-834, 1982.
DOI : 10.1093/brain/105.4.811

H. Mitsumoto and P. Gambetti, Impaired slow axonal transport in wobbler mouse motor neuron disease, Annals of Neurology, vol.91, issue.1, pp.36-43, 1986.
DOI : 10.1002/ana.410190108

N. Nakamura, C. Rabouille, R. Watson, T. Nilsson, N. Hui et al., Characterization of a cis-Golgi matrix protein, GM130, The Journal of Cell Biology, vol.131, issue.6, pp.1715-1726, 1995.
DOI : 10.1083/jcb.131.6.1715

R. Parvari, E. Hershkovitz, N. Grossman, R. Gorodischer, B. Loeys et al., Mutation of TBCE causes hypoparathyroidism???retardation???dysmorphism and autosomal recessive Kenny???Caffey syndrome, Nature Genetics, vol.27, issue.3, pp.448-452, 2002.
DOI : 10.1038/ng1012

R. Parvari, G. Diaz, and E. Hershkovitz, Parathyroid Development and the Role of Tubulin Chaperone E, Hormone Research in Paediatrics, vol.67, issue.1, pp.12-21, 2007.
DOI : 10.1159/000095944

P. Pasinelli and R. Brown, Molecular biology of amyotrophic lateral sclerosis: insights from genetics, Nature Reviews Neuroscience, vol.6, issue.2, pp.710-723, 2006.
DOI : 10.1038/nrn1971

F. Perrin, G. Boisset, A. Lathuiliere, and A. Kato, Cell death pathways differ in several mouse models with motoneurone disease: analysis of pure motoneurone populations at a presymptomatic age, Journal of Neurochemistry, vol.31, issue.6, pp.1959-1972, 2006.
DOI : 10.1111/j.1471-4159.2006.04024.x

I. Puls, C. Jonnakuty, B. Lamonte, E. Holzbaur, M. Tokito et al., Mutant dynactin in motor neuron disease, Nature Genetics, vol.33, issue.4, pp.455-456, 2003.
DOI : 10.1038/ng1123

S. Pun, A. Santos, S. Saxena, L. Xu, and P. Caroni, Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF, Nature Neuroscience, vol.11, issue.3, pp.408-419, 2006.
DOI : 10.1038/nn1653

C. Raoul, A. Estevez, H. Nishimune, D. Cleveland, O. Henderson et al., Motoneuron Death Triggered by a Specific Pathway Downstream of Fas, Neuron, vol.35, issue.6, pp.1067-1083, 2002.
DOI : 10.1016/S0896-6273(02)00905-4

S. Ravula, M. Wang, M. Mcclain, S. Asress, B. Frazier et al., Spatiotemporal localization of injury potentials in DRG neurons during vincristine-induced axonal degeneration, Neuroscience Letters, vol.415, issue.1, pp.34-39, 2007.
DOI : 10.1016/j.neulet.2007.01.009

F. Richmond, R. Gladdy, J. Creasy, S. Kitamura, E. Smits et al., Efficacy of seven retrograde tracers, compared in multiple-labelling studies of feline motoneurones, Journal of Neuroscience Methods, vol.53, issue.1, pp.35-46, 1994.
DOI : 10.1016/0165-0270(94)90142-2

R. Rios, A. Sanchis, A. Tassin, C. Fedriani, and M. Bornens, GMAP-210 Recruits ??-Tubulin Complexes to cis-Golgi Membranes and Is Required for Golgi Ribbon Formation, Cell, vol.118, issue.3, pp.323-335, 2004.
DOI : 10.1016/j.cell.2004.07.012

Y. Sagot, S. Tan, E. Baetge, H. Schmalbruch, A. Kato et al., Polymer Encapsulated Cell Lines Genetically Engineered to Release Ciliary Neurotrophic Factor Can Slow Down Progressive Motor Neuronopathy in the Mouse, European Journal of Neuroscience, vol.113, issue.6, pp.1313-1322, 1995.
DOI : 10.1111/j.1460-9568.1995.tb01122.x

Y. Sagot, M. Duboisdauphin, S. Tan, F. Debilbao, P. Aebischer et al., Bcl-2 overexpression prevents motoneuron cell body loss but not axonal degeneration in a mouse model of a neurodegenerative disease, J Neurosci, vol.15, pp.7727-7733, 1995.

S. Sapperstein, D. Walter, A. Grosvenor, J. Heuser, and M. Waters, p115 is a general vesicular transport factor related to the yeast endoplasmic reticulum to Golgi transport factor Uso1p., Proceedings of the National Academy of Sciences, vol.92, issue.2, pp.522-526, 1995.
DOI : 10.1073/pnas.92.2.522

H. Schmalbruch, H. Jensen, M. Bjaerg, Z. Kamieniecka, and L. Kurland, A New Mouse Mutant with Progressive Motor Neuronopathy, Journal of Neuropathology & Experimental Neurology, vol.50, issue.3, pp.192-204, 1991.
DOI : 10.1097/00005072-199105000-00002

T. Schmitt-john, C. Drepper, A. Mussmann, P. Hahn, M. Kuhlmann et al., Mutation of Vps54 causes motor neuron disease and defective spermiogenesis in the wobbler mouse, Nature Genetics, vol.105, issue.11, pp.1213-1215, 2005.
DOI : 10.1086/425287

J. Seemann, E. Jokitalo, M. Pypaert, and G. Warren, Matrix proteins can generate the higher order architecture of the Golgi apparatus, Nature, vol.407, pp.1022-1026, 2000.

M. Sendtner, H. Schmalbruch, K. Stöckli, P. Carroll, G. Kreutzberg et al., Ciliary neurotrophic factor prevents degeneration of motor neurons in mouse mutant progressive motor neuronopathy, Nature, vol.358, issue.6386, pp.502-504, 1992.
DOI : 10.1038/358502a0

A. Silva, Q. Wang, M. Wang, S. Ravula, and J. Glass, Evidence for direct axonal toxicity in vincristine neuropathy, Journal of the Peripheral Nervous System, vol.39, issue.3, pp.211-216, 2006.
DOI : 10.1002/ana.10300

Y. Simonin, F. Perrin, and A. Kato, Axonal involvement in the Wlds neuroprotective effect: analysis of pure motoneurons in a mouse model protected from motor neuron disease at a pre-symptomatic age, Journal of Neurochemistry, vol.122, issue.2, pp.530-542, 2007.
DOI : 10.1016/S0092-8674(01)00363-4

S. Terada, M. Kinjo, and N. Hirokawa, Oligomeric Tubulin in Large Transporting Complex Is Transported via Kinesin in Squid Giant Axons, Cell, vol.103, issue.1, pp.141-155, 2000.
DOI : 10.1016/S0092-8674(00)00094-5

G. Tian, Y. Huang, H. Rommelaere, J. Vandekerckhove, C. Ampe et al., Pathway Leading to Correctly Folded ??-Tubulin, Cell, vol.86, issue.2, pp.287-296, 1996.
DOI : 10.1016/S0092-8674(00)80100-2

G. Tian, S. Lewis, B. Feierbach, T. Stearns, H. Rommelaere et al., Tubulin Subunits Exist in an Activated Conformational State Generated and Maintained by Protein Cofactors, The Journal of Cell Biology, vol.124, issue.4, pp.821-832, 1997.
DOI : 10.1002/cm.970230306

G. Tian, M. Huang, R. Parvari, G. Diaz, and N. Cowan, Cryptic out-of-frame translational initiation of TBCE rescues tubulin formation in compound heterozygous HRD, Proceedings of the National Academy of Sciences, vol.103, issue.36, pp.13491-13496, 2006.
DOI : 10.1073/pnas.0602798103

W. Wang, J. Ding, E. Allen, P. Zhu, L. Zhang et al., Gigaxonin Interacts with Tubulin Folding Cofactor B and Controls Its Degradation through the Ubiquitin-Proteasome Pathway, Current Biology, vol.15, issue.22, pp.2050-2055, 2005.
DOI : 10.1016/j.cub.2005.10.052

D. Watson, P. Hoffman, and J. Griffin, The cold stability of microtubules increases during axonal maturation, J Neurosci, vol.10, pp.3344-3352, 1990.

K. Yamanaka, T. Miller, M. Mcalonis-downes, S. Chun, and D. Cleveland, Progressive spinal axonal degeneration and slowness in ALS2-deficient mice, Annals of Neurology, vol.575, issue.pt 2, pp.95-104, 2006.
DOI : 10.1002/ana.20888

W. Zenker and E. Hohberg, A-?-nerve-fibre: Number of neurotubules in the stem fibre and in the terminal branches, Journal of Neurocytology, vol.29, issue.2, pp.143-148, 1973.
DOI : 10.1007/BF01474716