H. Brahms, L. Meheus, V. De-brabandere, U. Fischer, and R. Luhrmann, Symmetrical dimethylation of arginine residues in spliceosomal Sm protein B/B??? and the Sm-like protein LSm4, and their interaction with the SMN protein, RNA, vol.7, issue.11, pp.1531-1542, 2001.
DOI : 10.1017/S135583820101442X

D. Buhler, V. Raker, R. Luhrmann, and U. Fischer, Essential Role for the Tudor Domain of SMN in Spliceosomal U snRNP Assembly: Implications for Spinal Muscular Atrophy, Human Molecular Genetics, vol.8, issue.13, pp.2351-2357, 1999.
DOI : 10.1093/hmg/8.13.2351

L. Campbell, K. M. Hunter, P. Mohaghegh, J. M. Tinsley, M. A. Brasch et al., Direct interaction of Smn with dp103, a putative RNA helicase: a role for Smn in transcription regulation?, Human Molecular Genetics, vol.9, issue.7, pp.1093-1100, 2000.
DOI : 10.1093/hmg/9.7.1093

B. Charroux, L. Pellizzoni, R. A. Perkinson, A. Shevchenko, M. Mann et al., Gemin3, The Journal of Cell Biology, vol.18, issue.6, pp.1181-1194, 1999.
DOI : 10.1128/MCB.18.2.676

B. Charroux, L. Pellizzoni, R. A. Perkinson, J. Yong, A. Shevchenko et al., Gemin4, The Journal of Cell Biology, vol.25, issue.6, pp.1177-1186, 2000.
DOI : 10.3109/10409239009090604

URL : http://jcb.rupress.org/content/jcb/148/6/1177.full.pdf

Y. D. Choi and G. Dreyfuss, Monoclonal antibody characterization of the C proteins of heterogeneous nuclear ribonucleoprotein complexes in vertebrate cells, The Journal of Cell Biology, vol.99, issue.6, pp.1997-2004, 1984.
DOI : 10.1083/jcb.99.6.1997

D. D. Coovert, T. T. Le, P. E. Mcandrew, J. Strasswimmer, T. O. Crawford et al., The survival motor neuron protein in spinal muscular atrophy, Human Molecular Genetics, vol.6, issue.8, pp.1205-1214, 1997.
DOI : 10.1093/hmg/6.8.1205

T. E. England and O. C. Uhlenbeck, 3???-Terminal labelling of RNA with T4 RNA ligase, Nature, vol.15, issue.5680, pp.560-561, 1978.
DOI : 10.1016/0092-8674(77)90130-1

U. Fischer, J. Huber, W. C. Boelens, I. W. Mattaj, and R. Luhrmann, The HIV-1 Rev Activation Domain is a nuclear export signal that accesses an export pathway used by specific cellular RNAs, Cell, vol.82, issue.3, pp.475-483, 1995.
DOI : 10.1016/0092-8674(95)90436-0

U. Fischer, Q. Liu, and G. Dreyfuss, The SMN???SIP1 Complex Has an Essential Role in Spliceosomal snRNP Biogenesis, Cell, vol.90, issue.6, pp.1023-1029, 1997.
DOI : 10.1016/S0092-8674(00)80368-2

U. Fischer and R. Luhrmann, An essential signaling role for the m3G cap in the transport of U1 snRNP to the nucleus, Science, vol.249, issue.4970, pp.786-790, 1990.
DOI : 10.1126/science.2143847

U. Fischer, V. Sumpter, M. Sekine, T. Satoh, and R. Luhrmann, Nucleo-cytoplasmic transport of U snRNPs: definition of a nuclear location signal in the Sm core domain that binds a transport receptor independently of the m3G cap, EMBO J, vol.12, pp.573-583, 1993.

M. Fornerod, M. Ohno, M. Yoshida, and I. W. Mattaj, CRM1 Is an Export Receptor for Leucine-Rich Nuclear Export Signals, Cell, vol.90, issue.6, pp.1051-1060, 1997.
DOI : 10.1016/S0092-8674(00)80371-2

URL : https://doi.org/10.1016/s0092-8674(00)80371-2

M. R. Frey and A. G. Matera, RNA-mediated interaction of Cajal bodies and U2 snRNA genes, The Journal of Cell Biology, vol.15, issue.3, pp.499-509, 2001.
DOI : 10.1017/S1355838299990726

URL : https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2196410/pdf

W. J. Friesen and G. Dreyfuss, Specific Sequences of the Sm and Sm-like (Lsm) Proteins Mediate Their Interaction with the Spinal Muscular Atrophy Disease Gene Product (SMN), Journal of Biological Chemistry, vol.11, issue.34, pp.26370-26375, 2000.
DOI : 10.1128/MCB.16.7.3668

W. J. Friesen, S. Massenet, S. Paushkin, A. Wyce, and G. Dreyfuss, SMN, the Product of the Spinal Muscular Atrophy Gene, Binds Preferentially to Dimethylarginine-Containing Protein Targets, Molecular Cell, vol.7, issue.5, pp.1111-1117, 2001.
DOI : 10.1016/S1097-2765(01)00244-1

W. J. Friesen, S. Paushkin, A. Wyce, S. Massenet, G. S. Pesiridis et al., The Methylosome, a 20S Complex Containing JBP1 and pICln, Produces Dimethylarginine-Modified Sm Proteins, Molecular and Cellular Biology, vol.21, issue.24, pp.8289-8300, 2001.
DOI : 10.1128/MCB.21.24.8289-8300.2001

URL : http://mcb.asm.org/content/21/24/8289.full.pdf

W. J. Friesen, A. Wyce, S. Paushkin, L. Abel, J. Rappsilber et al., A Novel WD Repeat Protein Component of the Methylosome Binds Sm Proteins, Journal of Biological Chemistry, vol.1381, issue.10, pp.8243-8247, 2002.
DOI : 10.1126/science.171.3971.579

T. Frugier, F. D. Tiziano, C. Cifuentes-diaz, P. Miniou, N. Roblot et al., Nuclear targeting defect of SMN lacking the C-terminus in a mouse model of spinal muscular atrophy, Human Molecular Genetics, vol.9, issue.5, pp.849-858, 2000.
DOI : 10.1093/hmg/9.5.849

A. C. Gilliam and J. A. Steitz, Rare scleroderma autoantibodies to the U11 small nuclear ribonucleoprotein and to the trimethylguanosine cap of U small nuclear RNAs., Proc. Natl. Acad. Sci. USA, pp.6781-6785, 1993.
DOI : 10.1073/pnas.90.14.6781

M. Gorlach, C. G. Burd, and G. Dreyfuss, The mRNA Poly(A)-Binding Protein: Localization, Abundance, and RNA-Binding Specificity, Experimental Cell Research, vol.211, issue.2, pp.400-407, 1994.
DOI : 10.1006/excr.1994.1104

D. Gorlich, R. Kraft, S. Kostka, F. Vogel, E. Hartmann et al., Importin Provides a Link between Nuclear Protein Import and U snRNA Export, Cell, vol.87, issue.1, pp.21-32, 1996.
DOI : 10.1016/S0092-8674(00)81319-7

A. K. Gubitz, Z. Mourelatos, L. Abel, J. Rappsilber, M. Mann et al., Gemin5, a Novel WD Repeat Protein Component of the SMN Complex That Binds Sm Proteins, Journal of Biological Chemistry, vol.15, issue.7, pp.5631-5636, 2002.
DOI : 10.1093/emboj/17.19.5577

J. Hamm, E. Darzynkiewicz, S. M. Tahara, and I. W. Mattaj, The trimethylguanosine cap structure of U1 snRNA is a component of a bipartite nuclear targeting signal, Cell, vol.62, issue.3, pp.569-577, 1990.
DOI : 10.1016/0092-8674(90)90021-6

J. Hamm and I. W. Mattaj, Monomethylated cap structures facilitate RNA export from the nucleus, Cell, vol.63, issue.1, pp.109-118, 1990.
DOI : 10.1016/0092-8674(90)90292-M

M. D. Hebert, P. W. Szymczyk, K. B. Shpargel, and A. G. Matera, Coilin forms the bridge between Cajal bodies and SMN, the Spinal Muscular Atrophy protein, Genes & Development, vol.15, issue.20, pp.2720-2729, 2001.
DOI : 10.1101/gad.908401

URL : http://genesdev.cshlp.org/content/15/20/2720.full.pdf

H. M. Hsieh-li, J. G. Chang, Y. J. Jong, M. H. Wu, N. M. Wang et al., A mouse model for spinal muscular atrophy, Nature Genetics, vol.227, issue.1, pp.66-70, 2000.
DOI : 10.1038/227680a0

R. Sekine and . Luhrmann, Snurportin1, an m3G-cap-specific nuclear import receptor with a novel domain structure, EMBO J, vol.17, pp.4114-4126, 1998.

E. Izaurralde, U. Kutay, C. Von-kobbe, I. W. Mattaj, and D. Gorlich, The asymmetric distribution of the constituents of the Ran system is essential for transport into and out of the nucleus, The EMBO Journal, vol.16, issue.21, pp.6535-6547, 1997.
DOI : 10.1093/emboj/16.21.6535

E. Izaurralde, J. Lewis, C. Gamberi, A. Jarmolowski, C. Mcguigan et al., A cap-binding protein complex mediating U snRNA export, Nature, vol.376, issue.6542, pp.709-712, 1995.
DOI : 10.1038/376709a0

E. Izaurralde, J. Lewis, C. Mcguigan, M. Jankowska, E. Darzynkiewicz et al., A nuclear cap binding protein complex involved in pre-mRNA splicing, Cell, vol.78, issue.4, pp.657-668, 1994.
DOI : 10.1016/0092-8674(94)90530-4

S. Jablonka, B. Schrank, M. Kralewski, W. Rossoll, and M. Sendtner, Reduced survival motor neuron (Smn) gene dose in mice leads to motor neuron degeneration: an animal model for spinal muscular atrophy type III, Human Molecular Genetics, vol.9, issue.3, pp.341-346, 2000.
DOI : 10.1093/hmg/9.3.341

A. Jarmolowski, W. C. Boelens, E. Izaurralde, and I. W. Mattaj, Nuclear export of different classes of RNA is mediated by specific factors, The Journal of Cell Biology, vol.124, issue.5, 1994.
DOI : 10.1083/jcb.124.5.627

C. Kambach, S. Walke, R. Young, J. M. Avis, E. De-la-fortelle et al., Crystal Structures of Two Sm Protein Complexes and Their Implications for the Assembly of the Spliceosomal snRNPs, Cell, vol.96, issue.3, pp.375-387, 1999.
DOI : 10.1016/S0092-8674(00)80550-4

N. Kataoka, M. Ohno, K. Kangawa, Y. Tokoro, and Y. Shimura, Cloning of a complementary DNA encoding an 80 kilodalton nuclear cap binding protein, Nucleic Acids Research, vol.22, issue.19, pp.3861-3865, 1994.
DOI : 10.1093/nar/22.19.3861

N. Kataoka, M. Ohno, I. Moda, and Y. Shimura, Identification of the factors that interact with NCBP, an 80 kDa nuclear cap binding protein, Nucleic Acids Research, vol.23, issue.18, pp.3638-3641, 1995.
DOI : 10.1093/nar/23.18.3638

M. R. Lerner and J. A. Steitz, Antibodies to small nuclear RNAs complexed with proteins are produced by patients with systemic lupus erythematosus, Proc. Natl. Acad. Sci. USA, pp.5495-5499, 1979.
DOI : 10.1073/pnas.76.11.5495

URL : http://www.pnas.org/content/76/11/5495.full.pdf

Q. Liu and G. Dreyfuss, A novel nuclear structure containing the survival of motor neurons protein, EMBO J, vol.15, pp.3555-3565, 1996.

Q. Liu, U. Fischer, F. Wang, and G. Dreyfuss, The Spinal Muscular Atrophy Disease Gene Product, SMN, and Its Associated Protein SIP1 Are in a Complex with Spliceosomal snRNP Proteins, Cell, vol.90, issue.6, pp.1013-1021, 1997.
DOI : 10.1016/S0092-8674(00)80367-0

I. W. Mattaj, Cap trimethylation of U snRNA is cytoplasmic and dependent on U snRNP protein binding, Cell, vol.46, issue.6, pp.905-911, 1986.
DOI : 10.1016/0092-8674(86)90072-3

I. W. Mattaj and E. M. De-robertis, Nuclear segregation of U2 snRNA requires binding of specific snRNP proteins, Cell, vol.40, issue.1, pp.111-118, 1985.
DOI : 10.1016/0092-8674(85)90314-9

G. Meister, D. Buhler, B. Laggerbauer, M. Zobawa, F. Lottspeich et al., Characterization of a nuclear 20S complex containing the survival of motor neurons (SMN) protein and a specific subset of spliceosomal Sm proteins, Human Molecular Genetics, vol.9, issue.13, pp.1977-1986, 2000.
DOI : 10.1093/hmg/9.13.1977

G. Meister, D. Buhler, R. Pillai, F. Lottspeich, and U. Fisher, A multiprotein complex mediates the ATP-dependent assembly of spliceosomal U snRNPs, Nature Cell Biology, vol.3, issue.11, pp.945-949, 2001.
DOI : 10.1038/ncb1101-945

G. Meister, C. Eggert, D. Buhler, H. Brahms, C. Kambach et al., Methylation of Sm proteins by a complex containing PRMT5 and the putative U snRNP assembly factor pICln, Current Biology, vol.11, issue.24, pp.1990-1994, 2001.
DOI : 10.1016/S0960-9822(01)00592-9

J. Melki, Spinal muscular atrophy, Current Opinion in Neurology, vol.10, issue.5, pp.381-385, 1997.
DOI : 10.1097/00019052-199710000-00005

U. R. Monani, M. Sendtner, D. D. Coovert, D. W. Parsons, C. Andreassi et al., The human centromeric survival motor neuron gene (SMN2) rescues embryonic lethality in Smn-/- mice and results in a mouse with spinal muscular atrophy, Human Molecular Genetics, vol.9, issue.3, pp.333-339, 2000.
DOI : 10.1093/hmg/9.3.333

J. Mouaikel, C. Verheggen, E. Bertrand, J. Tazi, and R. Bordonne, Hypermethylation of the Cap Structure of Both Yeast snRNAs and snoRNAs Requires a Conserved Methyltransferase that Is Localized to the Nucleolus, Molecular Cell, vol.9, issue.4, pp.891-901, 2002.
DOI : 10.1016/S1097-2765(02)00484-7

H. E. Neuman-de-vegvar and J. E. Dahlberg, Nucleocytoplasmic transport and processing of small nuclear RNA precursors., Molecular and Cellular Biology, vol.10, issue.7, pp.3365-3375, 1990.
DOI : 10.1128/MCB.10.7.3365

M. Ohno, A. Segref, A. Bachi, M. Wilm, and I. W. Mattaj, PHAX, a Mediator of U snRNA Nuclear Export Whose Activity Is Regulated by Phosphorylation, Cell, vol.101, issue.2, pp.187-198, 2000.
DOI : 10.1016/S0092-8674(00)80829-6

I. Palacios, M. Hetzer, S. A. Adam, and I. W. Mattaj, Nuclear import of U snRNPs requires importin beta, The EMBO Journal, vol.16, issue.22, pp.6783-6792, 1997.
DOI : 10.1093/emboj/16.22.6783

L. Pellizzoni, J. Baccon, B. Charroux, and G. Dreyfuss, The survival of motor neurons (SMN) protein interacts with the snoRNP proteins fibrillarin and GAR1, Current Biology, vol.11, issue.14, pp.1079-1088, 2001.
DOI : 10.1016/S0960-9822(01)00316-5

L. Pellizzoni, J. Baccon, J. Rappsilber, M. Mann, and G. Dreyfuss, Purification of Native Survival of Motor Neurons Complexes and Identification of Gemin6 as a Novel Component, Journal of Biological Chemistry, vol.15, issue.9, pp.7540-7545, 2002.
DOI : 10.1016/S0955-0674(00)00211-8

L. Pellizzoni, B. Charroux, and G. Dreyfuss, SMN mutants of spinal muscular atrophy patients are defective in binding to snRNP proteins, Proc. Natl. Acad. Sci. USA, pp.11167-11172, 1999.
DOI : 10.1073/pnas.78.5.2737

L. Pellizzoni, B. Charroux, J. Rappsilber, M. Mann, and G. Dreyfuss, A Functional Interaction between the Survival Motor Neuron Complex and RNA Polymerase II, The Journal of Cell Biology, vol.122, issue.1, pp.75-85, 2001.
DOI : 10.1093/emboj/16.6.1401

L. Pellizzoni, N. Kataoka, B. Charroux, and G. Dreyfuss, A Novel Function for SMN, the Spinal Muscular Atrophy Disease Gene Product, in Pre-mRNA Splicing, Cell, vol.95, issue.5, pp.615-624, 1998.
DOI : 10.1016/S0092-8674(00)81632-3

G. Plessel, U. Fischer, and R. Luhrmann, m3G cap hypermethylation of U1 small nuclear ribonucleoprotein (snRNP) in vitro: evidence that the U1 small nuclear RNA-(guanosine-N2)-methyltransferase is a non-snRNP cytoplasmic protein that requires a binding site on the Sm core domain., Molecular and Cellular Biology, vol.14, issue.6, pp.4160-4172, 1994.
DOI : 10.1128/MCB.14.6.4160

A. Segref, I. W. Mattaj, and M. Ohno, The evolutionarily conserved region of the U snRNA export mediator PHAX is a novel RNA-binding domain that is essential for U snRNA export, RNA, vol.7, issue.3, pp.351-360, 2001.
DOI : 10.1017/S1355838201002278

M. C. Siomi, P. S. Eder, N. Kataoka, L. Wan, Q. Liu et al., Transportin-mediated Nuclear Import of Heterogeneous Nuclear RNP Proteins, The Journal of Cell Biology, vol.81, issue.6, pp.1181-1192, 1997.
DOI : 10.1073/pnas.91.15.6924

URL : http://jcb.rupress.org/content/jcb/138/6/1181.full.pdf

C. L. Will and R. Luhrmann, Spliceosomal UsnRNP biogenesis, structure and function, Current Opinion in Cell Biology, vol.13, issue.3, pp.290-301, 2001.
DOI : 10.1016/S0955-0674(00)00211-8

J. Yong, L. Pellizzoni, and G. Dreyfuss, Sequence-specific interaction of U1 snRNA with the SMN complex, The EMBO Journal, vol.21, issue.5, pp.1188-1196, 2002.
DOI : 10.1093/emboj/21.5.1188