메뉴 건너뛰기




Volumn 125, Issue 11, 2012, Pages 2626-2637

Changes in intranuclear mobility of mature snrnps provide a mechanism for splicing defects in spinal muscular atrophy

Author keywords

Nuclear dynamics; Snrnps; Spinal muscular atrophy; Splicing speckles

Indexed keywords

MESSENGER RNA; SMALL NUCLEAR RIBONUCLEOPROTEIN; SURVIVAL MOTOR NEURON PROTEIN;

EID: 84865046210     PISSN: 00219533     EISSN: 14779137     Source Type: Journal    
DOI: 10.1242/jcs.096867     Document Type: Article
Times cited : (10)

References (63)
  • 1
    • 0017192686 scopus 로고
    • Mobility measurement by analysis of fluorescence photobleaching recovery kinetics
    • Axelrod, D., Koppel, D. E., Schlessinger, J., Elson, E. and Webb, W. W. (1976). Mobility measurement by analysis of fluorescence photobleaching recovery kinetics. Biophys. J. 16, 1055-1069.
    • (1976) Biophys. J. , vol.16 , pp. 1055-1069
    • Axelrod, D.1    Koppel, D.E.2    Schlessinger, J.3    Elson, E.4    Webb, W.W.5
  • 4
    • 78751685185 scopus 로고    scopus 로고
    • Impaired minor tri-snRNP assembly generates differential splicing defects of U12- type introns in lymphoblasts derived from a type I SMA patient
    • Boulisfane, N., Choleza, M., Rage, F., Neel, H., Soret, J. and Bordonné, R. (2011). Impaired minor tri-snRNP assembly generates differential splicing defects of U12- type introns in lymphoblasts derived from a type I SMA patient. Hum. Mol. Genet. 20, 641-648.
    • (2011) Hum. Mol. Genet. , vol.20 , pp. 641-648
    • Boulisfane, N.1    Choleza, M.2    Rage, F.3    Neel, H.4    Soret, J.5    Bordonné, R.6
  • 5
    • 77953122127 scopus 로고    scopus 로고
    • Specific splicing defects in S. pombe carrying a degron allele of the Survival of Motor Neuron gene
    • Campion, Y., Neel, H., Gostan, T., Soret, J. and Bordonné, R. (2010). Specific splicing defects in S. pombe carrying a degron allele of the Survival of Motor Neuron gene. EMBO J. 29, 1817-1829.
    • (2010) EMBO J , vol.29 , pp. 1817-1829
    • Campion, Y.1    Neel, H.2    Gostan, T.3    Soret, J.4    Bordonné, R.5
  • 6
    • 0033571601 scopus 로고    scopus 로고
    • The spinal muscular atrophy disease gene product SMN: A link between snRNP biogenesis and the Cajal (coiled) body
    • Carvalho, T., Almeida, F., Calapez, A., Lafarga, M., Berciano, M. T. and Carmo- Fonseca, M. (1999). The spinal muscular atrophy disease gene product, SMN: A link between snRNP biogenesis and the Cajal (coiled) body. J. Cell Biol. 147, 715-728
    • (1999) J. Cell Biol. , vol.147 , pp. 715-728
    • Carvalho, T.1    Almeida, F.2    Calapez, A.3    Lafarga, M.4    Berciano, M.T.5    Carmo-Fonseca, M.6
  • 7
    • 66049124365 scopus 로고    scopus 로고
    • The role of RNP biogenesis in spinal muscular atrophy
    • Chari, A., Paknia, E. and Fischer, U. (2009). The role of RNP biogenesis in spinal muscular atrophy. Curr. Opin. Cell Biol. 21, 387-393.
    • (2009) Curr. Opin. Cell Biol. , vol.21 , pp. 387-393
    • Chari, A.1    Paknia, E.2    Fischer, U.3
  • 8
    • 28444456713 scopus 로고    scopus 로고
    • Cajal bodies: a long history of discovery
    • Cioce, M. and Lamond, A. I. (2005). Cajal bodies: a long history of discovery. Annu. Rev. Cell Dev. Biol. 21, 105-131.
    • (2005) Annu. Rev. Cell Dev. Biol. , vol.21 , pp. 105-131
    • Cioce, M.1    Lamond, A.I.2
  • 9
    • 70350635179 scopus 로고    scopus 로고
    • The SMN protein is a key regulator of nuclear architecture in differentiating neuroblastoma cells
    • Clelland, A. K., Kinnear, N. P., Oram, L., Burza, J. and Sleeman, J. E. (2009). The SMN protein is a key regulator of nuclear architecture in differentiating neuroblastoma cells. Traffic 10, 1585-1598.
    • (2009) Traffic , vol.10 , pp. 1585-1598
    • Clelland, A.K.1    Kinnear, N.P.2    Oram, L.3    Burza, J.4    Sleeman, J.E.5
  • 10
    • 60149093432 scopus 로고    scopus 로고
    • RNA and disease
    • Cooper, T. A., Wan, L. and Dreyfuss, G. (2009). RNA and disease. Cell 136, 777-793
    • (2009) Cell , vol.136 , pp. 777-793
    • Cooper, T.A.1    Wan, L.2    Dreyfuss, G.3
  • 12
    • 45349106986 scopus 로고    scopus 로고
    • Spatial mapping of splicing factor complexes involved in exon and intron definition
    • Ellis, J. D., Llères, D., Denegri, M., Lamond, A. I. and Cáceres, J. F. (2008). Spatial mapping of splicing factor complexes involved in exon and intron definition. J. Cell Biol. 181, 921-934.
    • (2008) J. Cell Biol. , vol.181 , pp. 921-934
    • Ellis, J.D.1    Llères, D.2    Denegri, M.3    Lamond, A.I.4    Cáceres, J.F.5
  • 13
    • 0030924190 scopus 로고    scopus 로고
    • CRM1 is an export receptor for leucine-rich nuclear export signals
    • Fornerod, M., Ohno, M., Yoshida, M. and Mattaj, I. W. (1997). CRM1 is an export receptor for leucine-rich nuclear export signals. Cell 90, 1051-1060.
    • (1997) Cell , vol.90 , pp. 1051-1060
    • Fornerod, M.1    Ohno, M.2    Yoshida, M.3    Mattaj, I.W.4
  • 14
    • 60549104588 scopus 로고    scopus 로고
    • Splice-site pairing is an intrinsically high fidelity process
    • Fox-Walsh, K. L. and Hertel, K. J. (2009). Splice-site pairing is an intrinsically high fidelity process. Proc. Natl. Acad. Sci. USA 106, 1766-1771.
    • (2009) Proc. Natl. Acad. Sci. USA , vol.106 , pp. 1766-1771
    • Fox-Walsh, K.L.1    Hertel, K.J.2
  • 15
    • 41549119007 scopus 로고    scopus 로고
    • Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs
    • Gabanella, F., Butchbach, M. E., Saieva, L., Carissimi, C., Burghes, A. H. and Pellizzoni, L. (2007). Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs. PLoS ONE 2, e921.
    • (2007) PLoS ONE , vol.2
    • Gabanella, F.1    Butchbach, M.E.2    Saieva, L.3    Carissimi, C.4    Burghes, A.H.5    Pellizzoni, L.6
  • 16
    • 33745119863 scopus 로고    scopus 로고
    • Depletion of SMN by RNA interference in HeLa cells induces defects in Cajal body formation
    • Girard, C., Neel, H., Bertrand, E. and Bordonné, R. (2006). Depletion of SMN by RNA interference in HeLa cells induces defects in Cajal body formation. Nucleic Acids Res. 34, 2925-2932.
    • (2006) Nucleic Acids Res , vol.34 , pp. 2925-2932
    • Girard, C.1    Neel, H.2    Bertrand, E.3    Bordonné, R.4
  • 17
    • 34548356376 scopus 로고    scopus 로고
    • Two distinct arginine methyltransferases are required for biogenesis of Sm-class ribonucleoproteins
    • Gonsalvez, G. B., Tian, L., Ospina, J. K., Boisvert, F. M., Lamond, A. I. and Matera, A. G. (2007). Two distinct arginine methyltransferases are required for biogenesis of Sm-class ribonucleoproteins. J. Cell Biol. 178, 733-740.
    • (2007) J. Cell Biol. , vol.178 , pp. 733-740
    • Gonsalvez, G.B.1    Tian, L.2    Ospina, J.K.3    Boisvert, F.M.4    Lamond, A.I.5    Matera, A.G.6
  • 18
    • 0037017403 scopus 로고    scopus 로고
    • The importin-beta binding domain of snurportin1 is responsible for the Ran- and energy-independent nuclear import of spliceosomal U snRNPs in vitro
    • Huber, J., Dickmanns, A. and Lührmann, R. (2002). The importin-beta binding domain of snurportin1 is responsible for the Ran- and energy-independent nuclear import of spliceosomal U snRNPs in vitro. J. Cell Biol. 156, 467-479.
    • (2002) J. Cell Biol. , vol.156 , pp. 467-479
    • Huber, J.1    Dickmanns, A.2    Lührmann, R.3
  • 20
    • 0036900768 scopus 로고    scopus 로고
    • The interdependence of nuclear structure and function
    • Iborra, F. J. and Cook, P. R. (2002). The interdependence of nuclear structure and function. Curr. Opin. Cell Biol. 14, 780-785.
    • (2002) Curr. Opin. Cell Biol. , vol.14 , pp. 780-785
    • Iborra, F.J.1    Cook, P.R.2
  • 22
    • 0026751769 scopus 로고
    • Intracellular distribution of the U1A protein depends on active transport and nuclear binding to U1 snRNA
    • Kambach, C. and Mattaj, I. W. (1992). Intracellular distribution of the U1A protein depends on active transport and nuclear binding to U1 snRNA. J. Cell Biol. 118,11-21.
    • (1992) J. Cell Biol. , vol.118 , pp. 11-21
    • Kambach, C.1    Mattaj, I.W.2
  • 23
    • 0033524941 scopus 로고    scopus 로고
    • Crystal structures of two Sm protein complexes and their implications for the assembly of the spliceosomal snRNPs
    • Kambach, C., Walke, S., Young, R., Avis, J. M., de la Fortelle, E., Raker, V. A., Lührmann, R., Li, J. and Nagai, K. (1999). Crystal structures of two Sm protein complexes and their implications for the assembly of the spliceosomal snRNPs. Cell 96, 375-387.
    • (1999) Cell , vol.96 , pp. 375-387
    • Kambach, C.1    Walke, S.2    Young, R.3    Avis, J.M.4    de la Fortelle, E.5    Raker, V.A.6    Lührmann, R.7    Li, J.8    Nagai, K.9
  • 24
    • 0034631851 scopus 로고    scopus 로고
    • Reduced mobility of the alternate splicing factor (ASF) through the nucleoplasm and steady state speckle compartments
    • Kruhlak, M. J., Lever, M. A., Fischle, W., Verdin, E., Bazett-Jones, D. P. and Hendzel, M. J. (2000). Reduced mobility of the alternate splicing factor (ASF) through the nucleoplasm and steady state speckle compartments. J. Cell Biol. 150,41-52.
    • (2000) J. Cell Biol. , vol.150 , pp. 41-52
    • Kruhlak, M.J.1    Lever, M.A.2    Fischle, W.3    Verdin, E.4    Bazett-Jones, D.P.5    Hendzel, M.J.6
  • 30
    • 0029954338 scopus 로고    scopus 로고
    • A novel nuclear structure containing the survival of motor neurons protein
    • Liu, Q. and Dreyfuss, G. (1996). A novel nuclear structure containing the survival of motor neurons protein. EMBO J. 15, 3555-3565.
    • (1996) EMBO J , vol.15 , pp. 3555-3565
    • Liu, Q.1    Dreyfuss, G.2
  • 31
    • 22244432192 scopus 로고    scopus 로고
    • Evolutionary conservation of minor U12-type spliceosome between plants and humans
    • Lorković, Z. J., Lehner, R., Forstner, C. and Barta, A. (2005). Evolutionary conservation of minor U12-type spliceosome between plants and humans. RNA 11, 1095-1107.
    • (2005) RNA , vol.11 , pp. 1095-1107
    • Lorković, Z.J.1    Lehner, R.2    Forstner, C.3    Barta, A.4
  • 32
    • 0035889085 scopus 로고    scopus 로고
    • The concept of self-organization in cellular architecture
    • Misteli, T. (2001a). The concept of self-organization in cellular architecture. J. Cell Biol. 155, 181-186.
    • (2001) J. Cell Biol. , vol.155 , pp. 181-186
    • Misteli, T.1
  • 33
    • 0035793376 scopus 로고    scopus 로고
    • Protein dynamics: implications for nuclear architecture and gene expression
    • Misteli, T. (2001b). Protein dynamics: implications for nuclear architecture and gene expression. Science 291, 843-847.
    • (2001) Science , vol.291 , pp. 843-847
    • Misteli, T.1
  • 34
    • 78349288865 scopus 로고    scopus 로고
    • Higher-order genome organization in human disease
    • Misteli, T. (2010). Higher-order genome organization in human disease. Cold Spring Harb. Perspect. Biol. 2, a000794.
    • (2010) Cold Spring Harb. Perspect. Biol. , vol.2
    • Misteli, T.1
  • 35
    • 6344258807 scopus 로고    scopus 로고
    • Coupled in vitro import of U snRNPs and SMN, the spinal muscular atrophy protein
    • Narayanan, U., Achsel, T., Lührmann, R. and Matera, A. G. (2004). Coupled in vitro import of U snRNPs and SMN, the spinal muscular atrophy protein. Mol. Cell 16, 223-234.
    • (2004) Mol. Cell , vol.16 , pp. 223-234
    • Narayanan, U.1    Achsel, T.2    Lührmann, R.3    Matera, A.G.4
  • 37
    • 56649117675 scopus 로고    scopus 로고
    • The assembly of a spliceosomal small nuclear ribonucleoprotein particle
    • Patel, S. B. and Bellini, M. (2008). The assembly of a spliceosomal small nuclear ribonucleoprotein particle. Nucleic Acids Res. 36, 6482-6493.
    • (2008) Nucleic Acids Res , vol.36 , pp. 6482-6493
    • Patel, S.B.1    Bellini, M.2
  • 38
    • 34047148903 scopus 로고    scopus 로고
    • Chaperoning ribonucleoprotein biogenesis in health and disease
    • Pellizzoni, L. (2007). Chaperoning ribonucleoprotein biogenesis in health and disease. EMBO Rep. 8, 340-345.
    • (2007) EMBO Rep , vol.8 , pp. 340-345
    • Pellizzoni, L.1
  • 39
    • 0032567036 scopus 로고    scopus 로고
    • A novel function for SMN, the spinal muscular atrophy disease gene product, in pre-mRNA splicing
    • Pellizzoni, L., Kataoka, N., Charroux, B. and Dreyfuss, G. (1998). A novel function for SMN, the spinal muscular atrophy disease gene product, in pre-mRNA splicing. Cell 95, 615-624.
    • (1998) Cell , vol.95 , pp. 615-624
    • Pellizzoni, L.1    Kataoka, N.2    Charroux, B.3    Dreyfuss, G.4
  • 40
    • 0034611785 scopus 로고    scopus 로고
    • High mobility of proteins in the mammalian cell nucleus
    • Phair, R. D. and Misteli, T. (2000). High mobility of proteins in the mammalian cell nucleus. Nature 404, 604-609.
    • (2000) Nature , vol.404 , pp. 604-609
    • Phair, R.D.1    Misteli, T.2
  • 41
    • 68549111145 scopus 로고    scopus 로고
    • The spliceosome: a self-organized macromolecular machine in the nucleus
    • Rino, J.andCarmo-Fonseca, M.(2009). The spliceosome: a self-organized macromolecular machine in the nucleus? Trends Cell Biol. 19, 375-384.
    • (2009) Trends Cell Biol , vol.19 , pp. 375-384
    • Rino, J.1    andCarmo-Fonseca, M.2
  • 43
    • 78049354837 scopus 로고    scopus 로고
    • Spliceostatin A inhibits spliceosome assembly subsequent to prespliceosome formation
    • Roybal, G. A. and Jurica, M. S. (2010). Spliceostatin A inhibits spliceosome assembly subsequent to prespliceosome formation. Nucleic Acids Res. 38, 6664-6672.
    • (2010) Nucleic Acids Res , vol.38 , pp. 6664-6672
    • Roybal, G.A.1    Jurica, M.S.2
  • 44
    • 4143124637 scopus 로고    scopus 로고
    • RNAi knockdown of hPrp31 leads to an accumulation of U4/U6 di-snRNPs in Cajal bodies
    • Schaffert, N., Hossbach, M., Heintzmann, R., Achsel, T. and Lührmann, R. (2004). RNAi knockdown of hPrp31 leads to an accumulation of U4/U6 di-snRNPs in Cajal bodies. EMBO J. 23, 3000-3009.
    • (2004) EMBO J , vol.23 , pp. 3000-3009
    • Schaffert, N.1    Hossbach, M.2    Heintzmann, R.3    Achsel, T.4    Lührmann, R.5
  • 45
    • 28444454020 scopus 로고    scopus 로고
    • Gemin proteins are required for efficient assembly of Sm-class ribonucleoproteins
    • Shpargel, K. B. and Matera, A. G. (2005). Gemin proteins are required for efficient assembly of Sm-class ribonucleoproteins. Proc. Natl. Acad. Sci. USA 102, 17372-17377.
    • (2005) Proc. Natl. Acad. Sci. USA , vol.102 , pp. 17372-17377
    • Shpargel, K.B.1    Matera, A.G.2
  • 46
    • 34249752815 scopus 로고    scopus 로고
    • A regulatory role for CRM1 in the multi-directional trafficking of splicing snRNPs in the mammalian nucleus
    • Sleeman, J. (2007). A regulatory role for CRM1 in the multi-directional trafficking of splicing snRNPs in the mammalian nucleus. J. Cell Sci. 120, 1540-1550.
    • (2007) J. Cell Sci. , vol.120 , pp. 1540-1550
    • Sleeman, J.1
  • 47
    • 0033533730 scopus 로고    scopus 로고
    • Newly assembled snRNPs associate with coiled bodies before speckles, suggesting a nuclear snRNP maturation pathway
    • Sleeman, J. E. and Lamond, A. I. (1999). Newly assembled snRNPs associate with coiled bodies before speckles, suggesting a nuclear snRNP maturation pathway. Curr. Biol. 9, 1065-1074.
    • (1999) Curr. Biol. , vol.9 , pp. 1065-1074
    • Sleeman, J.E.1    Lamond, A.I.2
  • 48
    • 0035691790 scopus 로고    scopus 로고
    • snRNP protein expression enchances the formation of Cajal bodies containing p80 coilin and SMN
    • Sleeman, J. E., Ajuh, P. and Lamond, A. I. (2001). snRNP protein expression enchances the formation of Cajal bodies containing p80 coilin and SMN. J. Cell Sci. 114, 4407-4419.
    • (2001) J. Cell Sci. , vol.114 , pp. 4407-4419
    • Sleeman, J.E.1    Ajuh, P.2    Lamond, A.I.3
  • 49
    • 0038526320 scopus 로고    scopus 로고
    • Cajal body proteins SMN and Coilin show differential dynamic behaviour in vivo
    • Sleeman, J. E., Trinkle-Mulcahy, L., Prescott, A. R., Ogg, S. C. and Lamond, A. I. (2003). Cajal body proteins SMN and Coilin show differential dynamic behaviour in vivo. J. Cell Sci. 116, 2039-2050.
    • (2003) J. Cell Sci. , vol.116 , pp. 2039-2050
    • Sleeman, J.E.1    Trinkle-Mulcahy, L.2    Prescott, A.R.3    Ogg, S.C.4    Lamond, A.I.5
  • 51
    • 13244291467 scopus 로고    scopus 로고
    • FRAP analysis of binding: proper and fitting
    • Sprague, B. L. and McNally, J. G. (2005). FRAP analysis of binding: proper and fitting. Trends Cell Biol. 15, 84-91.
    • (2005) Trends Cell Biol , vol.15 , pp. 84-91
    • Sprague, B.L.1    McNally, J.G.2
  • 52
    • 0037450801 scopus 로고    scopus 로고
    • Targeting of U4/U6 small nuclear RNP assembly factor SART3/p110 to Cajal bodies
    • Stanek, D., Rader, S. D., Klingauf, M. and Neugebauer, K. M. (2003). Targeting of U4/U6 small nuclear RNP assembly factor SART3/p110 to Cajal bodies. J. Cell Biol. 160, 505-516.
    • (2003) J. Cell Biol. , vol.160 , pp. 505-516
    • Stanek, D.1    Rader, S.D.2    Klingauf, M.3    Neugebauer, K.M.4
  • 55
    • 79955832411 scopus 로고    scopus 로고
    • A missense mutation in PRPF6 causes impairment of pre-mRNA splicing and autosomal-dominant retinitis pigmentosa
    • Tanackovic, G., Ransijn, A., Ayuso, C., Harper, S., Berson, E. L. and Rivolta, C. (2011a). A missense mutation in PRPF6 causes impairment of pre-mRNA splicing and autosomal-dominant retinitis pigmentosa. Am. J. Hum. Genet. 88, 643-649.
    • (2011) Am. J. Hum. Genet. , vol.88 , pp. 643-649
    • Tanackovic, G.1    Ransijn, A.2    Ayuso, C.3    Harper, S.4    Berson, E.L.5    Rivolta, C.6
  • 56
    • 79955864211 scopus 로고    scopus 로고
    • PRPF mutations are associated with generalized defects in spliceosome formation and pre-mRNA splicing in patients with retinitis pigmentosa
    • Tanackovic, G., Ransijn, A., Thibault, P., Abou Elela, S., Klinck, R., Berson, E. L., Chabot, B. and Rivolta, C. (2011b). PRPF mutations are associated with generalized defects in spliceosome formation and pre-mRNA splicing in patients with retinitis pigmentosa. Hum. Mol. Genet. 20, 2116-2130.
    • (2011) Hum. Mol. Genet. , vol.20 , pp. 2116-2130
    • Tanackovic, G.1    Ransijn, A.2    Thibault, P.3    Abou Elela, S.4    Klinck, R.5    Berson, E.L.6    Chabot, B.7    Rivolta, C.8
  • 57
    • 0029863992 scopus 로고    scopus 로고
    • A novel spliceosome containing U11, U12, and U5 snRNPs excises a minor class (AT-AC) intron in vitro
    • Tarn, W. Y. and Steitz, J. A. (1996). A novel spliceosome containing U11, U12, and U5 snRNPs excises a minor class (AT-AC) intron in vitro. Cell 84, 801-811.
    • (1996) Cell , vol.84 , pp. 801-811
    • Tarn, W.Y.1    Steitz, J.A.2
  • 58
    • 60349104299 scopus 로고    scopus 로고
    • The spliceosome: design principles of a dynamic RNP machine
    • Wahl, M. C., Will, C. L. and Lührmann, R. (2009). The spliceosome: design principles of a dynamic RNP machine. Cell 136, 701-718.
    • (2009) Cell , vol.136 , pp. 701-718
    • Wahl, M.C.1    Will, C.L.2    Lührmann, R.3
  • 59
    • 20744455958 scopus 로고    scopus 로고
    • The survival of motor neurons protein determines the capacity for snRNP assembly: biochemical deficiency in spinal muscular atrophy
    • Wan, L., Battle, D. J., Yong, J., Gubitz, A. K., Kolb, S. J., Wang, J. and Dreyfuss, G. (2005). The survival of motor neurons protein determines the capacity for snRNP assembly: biochemical deficiency in spinal muscular atrophy. Mol. Cell. Biol. 25, 5543-5551.
    • (2005) Mol. Cell. Biol. , vol.25 , pp. 5543-5551
    • Wan, L.1    Battle, D.J.2    Yong, J.3    Gubitz, A.K.4    Kolb, S.J.5    Wang, J.6    Dreyfuss, G.7
  • 60
    • 0035370526 scopus 로고    scopus 로고
    • Spliceosomal UsnRNP biogenesis, structure and function
    • Will, C. L. and Lührmann, R. (2001). Spliceosomal UsnRNP biogenesis, structure and function. Curr. Opin. Cell Biol. 13, 290-301.
    • (2001) Curr. Opin. Cell Biol. , vol.13 , pp. 290-301
    • Will, C.L.1    Lührmann, R.2
  • 61
    • 0037634375 scopus 로고    scopus 로고
    • Identification of both shared and distinct proteins in the major and minor spliceosomes
    • Will, C. L., Schneider, C., Reed, R. and Lührmann, R. (1999). Identification of both shared and distinct proteins in the major and minor spliceosomes. Science 284, 2003-2005
    • (1999) Science , vol.284 , pp. 2003-2005
    • Will, C.L.1    Schneider, C.2    Reed, R.3    Lührmann, R.4
  • 62
    • 0034194444 scopus 로고    scopus 로고
    • The relationship between SMN, the spinal muscular atrophy protein, and nuclear coiled bodies in differentiated tissues and cultured cells
    • Young, P. J., Le, T. T., thi Man, N., Burghes, A. H. and Morris, G. E. (2000). The relationship between SMN, the spinal muscular atrophy protein, and nuclear coiled bodies in differentiated tissues and cultured cells. Exp. Cell Res. 256, 365-374.
    • (2000) Exp. Cell Res. , vol.256 , pp. 365-374
    • Young, P.J.1    Le, T.T.2    thi Man, N.3    Burghes, A.H.4    Morris, G.E.5
  • 63
    • 43049168361 scopus 로고    scopus 로고
    • SMN deficiency causes tissue-specific perturbations in the repertoire of snRNAs and widespread defects in splicing
    • Zhang, Z., Lotti, F., Dittmar, K., Younis, I., Wan, L., Kasim, M. and Dreyfuss, G. (2008). SMN deficiency causes tissue-specific perturbations in the repertoire of snRNAs and widespread defects in splicing. Cell 133, 585-600.
    • (2008) Cell , vol.133 , pp. 585-600
    • Zhang, Z.1    Lotti, F.2    Dittmar, K.3    Younis, I.4    Wan, L.5    Kasim, M.6    Dreyfuss, G.7


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.