R. Adalbert, ) with improved preservation of neuromuscular synapses, European Journal of Neuroscience, vol.39, issue.1, pp.271-277, 2005.
DOI : 10.1111/j.1460-9568.2004.03833.x

M. B. Alonso, Identification and Characterization of ZFP-57, a Novel Zinc Finger Transcription Factor in the Mammalian Peripheral Nervous System, Journal of Biological Chemistry, vol.279, issue.24, pp.25653-25664, 2004.
DOI : 10.1074/jbc.M400415200

R. M. Anderson, K. J. Bitterman, J. G. Wood, O. Medvedik, H. Cohen et al., Manipulation of a Nuclear NAD+ Salvage Pathway Delays Aging without Altering Steady-state NAD+ Levels, Journal of Biological Chemistry, vol.277, issue.21, pp.18881-18890, 2002.
DOI : 10.1074/jbc.M111773200

T. Araki, Y. Sasaki, and J. Milbrandt, Increased Nuclear NAD Biosynthesis and SIRT1 Activation Prevent Axonal Degeneration, Science, vol.305, issue.5686, pp.1010-1013, 2004.
DOI : 10.1126/science.1098014

P. Blume-jensen and T. Hunter, Oncogenic kinase signalling, Nature, vol.411, issue.6835, pp.355-365, 2001.
DOI : 10.1038/35077225

R. K. Campbell, E. R. Bergert, Y. Wang, J. C. Morris, and W. R. Moyle, Chimeric proteins can exceed the sum of their parts: Implications for evolution and protein design, Nature Biotechnology, vol.10, issue.5, pp.439-443, 1997.
DOI : 10.1038/nbt0597-439

M. P. 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

M. P. Coleman, P. , and V. H. , Axon pathology in neurological disease: a neglected therapeutic target, Trends in Neurosciences, vol.25, issue.10, pp.532-537, 2002.
DOI : 10.1016/S0166-2236(02)02255-5

M. P. Coleman and R. R. Ribchester, Programmed Axon Death, Synaptic Dysfunction and the Ubiquitin Proteasome System, Current Drug Target -CNS & Neurological Disorders, vol.3, issue.3, pp.227-238, 2004.
DOI : 10.2174/1568007043337436

L. Conforti, A. Tarlton, T. G. Mack, W. Mi, E. A. Buckmaster et al., A Ufd2/D4Cole1e chimeric protein and overexpression of Rbp7 in the slow Wallerian degeneration (WldS) mouse, Proc. Natl. Acad. Sci. USA 97, pp.11377-11382, 2000.
DOI : 10.1073/pnas.97.21.11377

T. O. Crawford, S. T. Hsieh, B. L. Schryer, and J. D. Glass, Prolonged axonal survival in transected nerves of C57BL/Ola mice is independent of age, Journal of Neurocytology, vol.246, issue.5, pp.333-340, 1995.
DOI : 10.1007/BF01189060

R. M. Dai, E. Chen, D. L. Longo, C. M. Gorbea, L. et al., Involvement of Valosin-containing Protein, an ATPase Co-purified with Ikappa Balpha and 26 S Proteasome, in Ubiquitin-Proteasome-mediated Degradation of Ikappa Balpha, Journal of Biological Chemistry, vol.273, issue.6, pp.3562-3573, 1998.
DOI : 10.1074/jbc.273.6.3562

R. M. Dai, L. , and C. C. , Valosin-containing protein is a multi-ubiquitin chain-targeting factor required in ubiquitin-proteasome degradation, Nature Cell Biology, vol.3, issue.8, pp.740-744, 2001.
DOI : 10.1038/35087056

M. Emanuelli, F. Carnevali, F. Saccucci, F. Pierella, A. Amici et al., Molecular Cloning, Chromosomal Localization, Tissue mRNA Levels, Bacterial Expression, and Enzymatic Properties of Human NMN Adenylyltransferase, Journal of Biological Chemistry, vol.276, issue.1, pp.406-412, 2001.
DOI : 10.1074/jbc.M008700200

A. Ferri, J. R. Sanes, M. P. Coleman, J. M. Cunningham, and A. C. 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

J. Fujimoto, M. Shiota, T. Iwahara, N. Seki, H. Satoh et al., Characterization of the transforming activity of p80, a hyperphosphorylated protein in a Ki-1 lymphoma cell line with chromosomal translocation t(2;5)., Proc. Natl. Acad. Sci. USA 93, pp.4181-4186, 1996.
DOI : 10.1073/pnas.93.9.4181

R. G. Gardner, Z. W. Nelson, and D. E. Gottschling, Degradation-Mediated Protein Quality Control in the Nucleus, Cell, vol.120, issue.6, pp.803-815, 2005.
DOI : 10.1016/j.cell.2005.01.016

S. Hatakeyama, M. Yada, M. Matsumoto, N. Ishida, and K. I. Nakayama, U Box Proteins as a New Family of Ubiquitin-Protein Ligases, Journal of Biological Chemistry, vol.276, issue.35, pp.32111-33120, 2001.
DOI : 10.1074/jbc.M102755200

C. E. Henderson, E. Bloch-gallego, and W. Camu, Purified embryonic motoneurons In: Nerve Cell Culture: A Practical Approach, pp.69-81, 1995.

M. Hetzer, H. H. Meyer, T. C. Walther, D. Bilbao-cortes, G. Warren et al., Distinct AAA-ATPase p97 complexes function in discrete steps of nuclear assembly, Nature Cell Biology, vol.3, issue.12, pp.1086-1091, 2001.
DOI : 10.1038/ncb1201-1086

H. Higashiyama, F. Hirose, M. Yamaguchi, Y. H. Inoue, N. Fujikake et al., Identification of ter94, Drosophila VCP, as a modulator of polyglutamine-induced neurodegeneration, Cell Death and Differentiation, vol.120, issue.3, pp.264-273, 2002.
DOI : 10.1038/sj.cdd.4400955

M. Hirabayashi, VCP/p97 in abnormal protein aggregates, cytoplasmic vacuoles, and cell death, phenotypes relevant to neurodegeneration, Cell Death and Differentiation, vol.8, issue.10, pp.977-984, 2001.
DOI : 10.1038/sj.cdd.4400907

T. Hoppe, Multiubiquitylation by E4 enzymes: ???one size??? doesn't fit all, Trends in Biochemical Sciences, vol.30, issue.4, pp.183-187, 2005.
DOI : 10.1016/j.tibs.2005.02.004

T. Hoppe, G. Cassata, J. M. Barral, W. Springer, A. H. Hutagalung et al., Regulation of the Myosin-Directed Chaperone UNC-45 by a Novel E3/E4-Multiubiquitylation Complex in C. elegans, Cell, vol.118, issue.3, pp.337-349, 2004.
DOI : 10.1016/j.cell.2004.07.014

F. E. Indig, J. J. Partridge, K. Cv, C. Aladjem, M. I. Latterich et al., Werner syndrome protein directly binds to the AAA ATPase p97/VCP in an ATP-dependent fashion, Journal of Structural Biology, vol.146, issue.1-2, pp.251-259, 2004.
DOI : 10.1016/j.jsb.2003.11.009

S. Ishigaki, N. Hishikawa, J. Niwa, S. Iemura, T. Natsume et al., Physical and Functional Interaction between Dorfin and Valosin-containing Protein That Are Colocalized in Ubiquitylated Inclusions in Neurodegenerative Disorders, Journal of Biological Chemistry, vol.279, issue.49, pp.51376-51385, 2004.
DOI : 10.1074/jbc.M406683200

E. Jarosch, C. Taxis, C. Volkwein, J. Bordallo, D. Finley et al., Protein dislocation from the ER requires polyubiquitination and the AAA-ATPase Cdc48, Nature Cell Biology, vol.4, issue.2, pp.134-139, 2002.
DOI : 10.1038/ncb746

C. Kaneko, S. Hatakeyama, M. Matsumoto, M. Yada, K. Nakayama et al., Characterization of the mouse gene for the U-box-type ubiquitin ligase UFD2a, Biochemical and Biophysical Research Communications, vol.300, issue.2, pp.297-304, 2003.
DOI : 10.1016/S0006-291X(02)02834-6

T. Kobayashi, K. Tanaka, K. Inoue, and A. Kakizuka, Functional ATPase Activity of p97/Valosin-containing Protein (VCP) Is Required for the Quality Control of Endoplasmic Reticulum in Neuronally Differentiated Mammalian PC12 Cells, Journal of Biological Chemistry, vol.277, issue.49, pp.47358-47365, 2002.
DOI : 10.1074/jbc.M207783200

M. Koegl, T. Hoppe, S. Schlenker, H. D. Ulrich, T. U. Mayer et al., A Novel Ubiquitination Factor, E4, Is Involved in Multiubiquitin Chain Assembly, Cell, vol.96, issue.5, pp.635-644, 1999.
DOI : 10.1016/S0092-8674(00)80574-7

H. Kondo, C. Rabouille, R. Newman, T. P. Levine, D. Pappin et al., p47 is a cofactor for p97-mediated membrane fusion, Nature, vol.388, pp.75-78, 1997.

C. Krona, K. Ejeskar, F. Abel, P. Kogner, J. Bjelke et al., Screening for gene mutations in a 500???kb neuroblastoma tumor suppressor candidate region in chromosome 1p; mutation and stage-specific expression in UBE4B/UFD2, Oncogene, vol.22, issue.15, pp.2343-2351, 2003.
DOI : 10.1038/sj.onc.1206324

B. L. Macinnis, C. , and R. B. , Regulation of Wallerian degeneration and nerve growth factor withdrawal-induced pruning of axons of sympathetic neurons by the proteasome and the MEK/Erk pathway, Molecular and Cellular Neuroscience, vol.28, issue.3, pp.430-439, 2005.
DOI : 10.1016/j.mcn.2004.10.003

T. G. Mack, Wallerian degeneration of injured axons and synapses is delayed by a Ube4b/Nmnat chimeric gene, Nature Neuroscience, vol.4, issue.12, pp.1199-1206, 2001.
DOI : 10.1038/nn770

G. Magni, A. Amici, M. Emanuelli, G. Orsomando, N. Raffaelli et al., Structure and Function of Nicotinamide Mononucleotide Adenylyltransferase, Current Medicinal Chemistry, vol.11, issue.7, pp.873-885, 2004.
DOI : 10.2174/0929867043455666

J. A. Mahoney, J. A. Odin, S. M. White, D. Shaffer, A. Koff et al., The human homologue of the yeast polyubiquitination factor Ufd2p is cleaved by caspase 6 and granzyme B during apoptosis, Biochemical Journal, vol.361, issue.3, pp.587-595, 2002.
DOI : 10.1042/bj3610587

F. Marti and P. D. King, The p95-100kDa ligand of the T cell-specific adaptor (TSAd) protein Src-homology-2 (SH2) domain implicated in TSAd nuclear import is p97 Valosin-containing protein (VCP), Immunology Letters, vol.97, issue.2, pp.235-243, 2005.
DOI : 10.1016/j.imlet.2004.10.021

M. Matsumoto, M. Yada, S. Hatakeyama, H. Ishimoto, T. Tanimura et al., Molecular clearance of ataxin-3 is regulated by a mammalian E4, The EMBO Journal, vol.23, issue.3, pp.659-669, 2004.
DOI : 10.1038/sj.emboj.7600081

H. H. Meyer, J. G. Shorter, J. Seemann, D. Pappin, W. et al., A complex of mammalian Ufd1 and Npl4 links the AAA-ATPase, p97, to ubiquitin and nuclear transport pathways, The EMBO Journal, vol.96, issue.10, pp.2181-2192, 2000.
DOI : 10.1093/emboj/19.10.2181

W. Mi, B. Beirowski, T. H. Gillingwater, R. Adalbert, D. Wagner et al., The slow Wallerian degeneration gene, WldS, inhibits axonal spheroid pathology in gracile axonal dystrophy mice, Brain, vol.128, issue.2, pp.405-416, 2005.
DOI : 10.1093/brain/awh368

Y. Mizuno, S. Hori, A. Kakizuka, and K. Okamoto, Vacuole-creating protein in neurodegenerative diseases in humans, Neuroscience Letters, vol.343, issue.2, pp.77-80, 2003.
DOI : 10.1016/S0304-3940(03)00280-5

A. Mogk, D. Dougan, J. Weibezahn, C. Schlieker, K. Turgay et al., Broad yet high substrate specificity: the challenge of AAA+ proteins, Journal of Structural Biology, vol.146, issue.1-2, 2004.
DOI : 10.1016/j.jsb.2003.10.009

M. Muratani and W. P. Tansey, How the ubiquitin???proteasome system controls transcription, Nature Reviews Molecular Cell Biology, vol.282, issue.3, pp.192-201, 2003.
DOI : 10.1038/nrm1049

F. Okumura, S. Hatakeyama, M. Matsumoto, T. Kamura, and K. I. Nakayama, Functional Regulation of FEZ1 by the U-box-type Ubiquitin Ligase E4B Contributes to Neuritogenesis, Journal of Biological Chemistry, vol.279, issue.51, pp.53533-53543, 2004.
DOI : 10.1074/jbc.M402916200

E. Rabinovich, A. Kerem, K. U. Frohlich, N. Diamant, and S. Bar-nun, AAA-ATPase p97/Cdc48p, a Cytosolic Chaperone Required for Endoplasmic Reticulum-Associated Protein Degradation, Molecular and Cellular Biology, vol.22, issue.2, pp.626-634, 2002.
DOI : 10.1128/MCB.22.2.626-634.2002

N. Raffaelli, L. Sorci, A. Amici, M. Emanuelli, F. Mazzola et al., Identification of a novel human nicotinamide mononucleotide adenylyltransferase, Biochemical and Biophysical Research Communications, vol.297, issue.4, pp.835-840, 2002.
DOI : 10.1016/S0006-291X(02)02285-4

C. Raoul, A. G. Estevez, H. Nishimune, D. W. Cleveland, O. Delapeyriere 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

H. Richly, M. Rape, S. Braun, S. Rumpf, C. Hoege et al., A Series of Ubiquitin Binding Factors Connects CDC48/p97 to Substrate Multiubiquitylation and Proteasomal Targeting, Cell, vol.120, issue.1, pp.73-84, 2005.
DOI : 10.1016/j.cell.2004.11.013

A. Sajadi, B. L. Schneider, and P. Aebischer, Wlds-Mediated Protection of Dopaminergic Fibers in an Animal Model of Parkinson Disease, Current Biology, vol.14, issue.4, pp.326-330, 2004.
DOI : 10.1016/j.cub.2004.01.053

M. Samsam, W. Mi, C. Wessig, J. Zielasek, K. V. Toyka et al., The Wlds mutation delays robust loss of motor and sensory axons in a genetic model for myelin-related axonopathy, J. Neurosci, vol.23, pp.2833-2839, 2003.

R. Schroder, G. D. Watts, S. G. Mehta, B. O. Evert, P. Broich et al., Mutant valosin-containing protein causes a novel type of frontotemporal dementia, Annals of Neurology, vol.24, issue.3, pp.457-461, 2005.
DOI : 10.1002/ana.20407

S. Spinette, C. Lengauer, J. A. Mahoney, P. V. Jallepalli, Z. Wang et al., Ufd2, a Novel Autoantigen in Scleroderma, Regulates Sister Chromatid Separation, Cell Cycle, vol.3, issue.12, pp.1638-1644, 2004.
DOI : 10.4161/cc.3.12.1345

J. Wang, Q. Zhai, Y. Chen, E. Lin, W. Gu et al., A local mechanism mediates NAD-dependent protection of axon degeneration, The Journal of Cell Biology, vol.294, issue.3, pp.349-355, 2005.
DOI : 10.1101/gad.1199904

M. S. Wang, A. A. Davis, D. G. Culver, and J. D. Glass, Wlds mice are resistant to paclitaxel (taxol) neuropathy, Annals of Neurology, vol.35, issue.4, pp.442-447, 2002.
DOI : 10.1002/ana.10300

Q. Wang, C. Song, L. , and C. C. , Molecular perspectives on p97???VCP: progress in understanding its structure and diverse biological functions, Journal of Structural Biology, vol.146, issue.1-2, pp.44-57, 2004.
DOI : 10.1016/j.jsb.2003.11.014

G. D. Watts, J. Wymer, M. J. Kovach, S. G. Mehta, S. Mumm et al., Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein, Nature Genetics, vol.36, issue.4, pp.377-381, 2004.
DOI : 10.1038/ng1332

C. Wojcik, M. Yano, and G. N. Demartino, RNA interference of valosin-containing protein (VCP/p97) reveals multiple cellular roles linked to ubiquitin/proteasome-dependent proteolysis, Journal of Cell Science, vol.117, issue.2, pp.281-292, 2004.
DOI : 10.1242/jcs.00841

R. Wu, A. V. Terry, P. B. Singh, G. , and D. M. , Differential Subnuclear Localization and Replication Timing of Histone H3 Lysine 9 Methylation States, Molecular Biology of the Cell, vol.16, issue.6, pp.2872-2881, 2005.
DOI : 10.1091/mbc.E04-11-0997

K. Yamanaka, Y. Okubo, T. Suzaki, and T. Ogura, Analysis of the two p97/VCP/Cdc48p proteins of Caenorhabditis elegans and their suppression of polyglutamine-induced protein aggregation, Journal of Structural Biology, vol.146, issue.1-2, pp.242-250, 2004.
DOI : 10.1016/j.jsb.2003.11.017

Q. Zhai, J. Wang, A. Kim, Q. Liu, R. Watts et al., Involvement of the Ubiquitin-Proteasome System in the Early Stages of Wallerian Degeneration, Neuron, vol.39, issue.2, pp.217-225, 2003.
DOI : 10.1016/S0896-6273(03)00429-X