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Volumn 34, Issue 3, 2012, Pages 213-222

Valproic acid increases SMN2 expression and modulates SF2/ASF and hnRNPA1 expression in SMA fibroblast cell lines

Author keywords

[No Author keywords available]

Indexed keywords

HETEROGENEOUS NUCLEAR RIBONUCLEOPROTEIN; HETEROGENEOUS NUCLEAR RIBONUCLEOPROTEIN A1; SPLICING FACTOR 2; SURVIVAL MOTOR NEURON PROTEIN 2; TRANSCRIPTION FACTOR; UNCLASSIFIED DRUG; VALPROIC ACID;

EID: 84856962413     PISSN: 03877604     EISSN: 18727131     Source Type: Journal    
DOI: 10.1016/j.braindev.2011.04.010     Document Type: Article
Times cited : (33)

References (58)
  • 1
    • 0036154959 scopus 로고    scopus 로고
    • Quantitative analyses of SMN1 and SMN2 based on real-time LightCycler PCR: fast and highly reliable carrier testing and prediction of severity of spinal muscular atrophy
    • Feldkötter M., Schwarzer V., Wirth R., Wienker T.F., Wirth B. Quantitative analyses of SMN1 and SMN2 based on real-time LightCycler PCR: fast and highly reliable carrier testing and prediction of severity of spinal muscular atrophy. Am J Hum Genet 2002, 70:358-368.
    • (2002) Am J Hum Genet , vol.70 , pp. 358-368
    • Feldkötter, M.1    Schwarzer, V.2    Wirth, R.3    Wienker, T.F.4    Wirth, B.5
  • 2
    • 0032991013 scopus 로고    scopus 로고
    • 59th ENMC International Workshop: Spinal Muscular Atrophies: recent progress and revised diagnostic criteria 17-19 April 1998, Soestduinen, The Netherlands
    • Zerres K., Davies K.E. 59th ENMC International Workshop: Spinal Muscular Atrophies: recent progress and revised diagnostic criteria 17-19 April 1998, Soestduinen, The Netherlands. Neuromuscul Disord 1999, 9:272-278.
    • (1999) Neuromuscul Disord , vol.9 , pp. 272-278
    • Zerres, K.1    Davies, K.E.2
  • 5
    • 0025319713 scopus 로고
    • Gene for chronic proximal spinal muscular atrophies maps to chromosome 5q
    • Melki J., Abdelhak S., Sheth P., Bachelot M.F., Burlet P., Marcadet A., et al. Gene for chronic proximal spinal muscular atrophies maps to chromosome 5q. Nature 1990, 344:767-768.
    • (1990) Nature , vol.344 , pp. 767-768
    • Melki, J.1    Abdelhak, S.2    Sheth, P.3    Bachelot, M.F.4    Burlet, P.5    Marcadet, A.6
  • 6
    • 0028797783 scopus 로고
    • Identification and characterization of a spinal muscular atrophy-determining gene
    • Lefebvre S., Bürglen L., Reboullet S., Clermont O., Burlet P., Viollet L., et al. Identification and characterization of a spinal muscular atrophy-determining gene. Cell 1995, 80:155-165.
    • (1995) Cell , vol.80 , pp. 155-165
    • Lefebvre, S.1    Bürglen, L.2    Reboullet, S.3    Clermont, O.4    Burlet, P.5    Viollet, L.6
  • 7
    • 0030981541 scopus 로고    scopus 로고
    • Correlation between severity and SMN protein level in spinal muscular atrophy
    • Lefebvre S., Burlet P., Liu Q., Bertrandy S., Clermont O., Munnich A., et al. Correlation between severity and SMN protein level in spinal muscular atrophy. Nat Genet 1997, 16:265-269.
    • (1997) Nat Genet , vol.16 , pp. 265-269
    • Lefebvre, S.1    Burlet, P.2    Liu, Q.3    Bertrandy, S.4    Clermont, O.5    Munnich, A.6
  • 8
    • 0034007548 scopus 로고    scopus 로고
    • An update of the mutation spectrum of the survival motor neuron gene (SMN1) in autosomal recessive spinal muscular atrophy (SMA)
    • Wirth B. An update of the mutation spectrum of the survival motor neuron gene (SMN1) in autosomal recessive spinal muscular atrophy (SMA). Hum Mutat 2000, 15:228-237.
    • (2000) Hum Mutat , vol.15 , pp. 228-237
    • Wirth, B.1
  • 9
    • 0028842926 scopus 로고
    • A frame-shift deletion in the survival motor neuron gene in Spanish spinal muscular atrophy patients
    • Bussaglia E., Clermont O., Tizzano E., Lefebvre S., Bürglen L., Cruaud C., et al. A frame-shift deletion in the survival motor neuron gene in Spanish spinal muscular atrophy patients. Nat Genet 1995, 11:335-337.
    • (1995) Nat Genet , vol.11 , pp. 335-337
    • Bussaglia, E.1    Clermont, O.2    Tizzano, E.3    Lefebvre, S.4    Bürglen, L.5    Cruaud, C.6
  • 11
    • 0032471510 scopus 로고    scopus 로고
    • Intragenic telSMN mutations: frequency, distribution, evidence of a founder effect, and modification of the spinal muscular atrophy phenotype by cenSMN copy number
    • Parsons D.W., McAndrew P.E., Iannaccone S.T., Mendell J.R., Burghes A.H.M., Prior T.W. Intragenic telSMN mutations: frequency, distribution, evidence of a founder effect, and modification of the spinal muscular atrophy phenotype by cenSMN copy number. Am J Hum Genet 1998, 63:1712-1723.
    • (1998) Am J Hum Genet , vol.63 , pp. 1712-1723
    • Parsons, D.W.1    McAndrew, P.E.2    Iannaccone, S.T.3    Mendell, J.R.4    Burghes, A.H.M.5    Prior, T.W.6
  • 12
    • 0036042048 scopus 로고    scopus 로고
    • Correlation between SMN2 copy number and clinical phenotype of spinal muscular atrophy: three SMN2 copies fail to rescue some patients from the disease severity
    • Harada Y., Sutomo R., Sadewa A.H., Akutsu T., Takeshima Y., Wada H., et al. Correlation between SMN2 copy number and clinical phenotype of spinal muscular atrophy: three SMN2 copies fail to rescue some patients from the disease severity. J Neurol 2002, 249:1211-1219.
    • (2002) J Neurol , vol.249 , pp. 1211-1219
    • Harada, Y.1    Sutomo, R.2    Sadewa, A.H.3    Akutsu, T.4    Takeshima, Y.5    Wada, H.6
  • 14
    • 0032799998 scopus 로고    scopus 로고
    • A single nucleotide difference that alters splicing patterns distinguishes the SMA gene SMN1 from the copy gene SMN2
    • Monani U.R., Lorson C.L., Parsons D.W., Prior T.W., Androphy E.J., Burghes A.H.M., et al. A single nucleotide difference that alters splicing patterns distinguishes the SMA gene SMN1 from the copy gene SMN2. Hum Mol Genet 1999, 8:1177-1183.
    • (1999) Hum Mol Genet , vol.8 , pp. 1177-1183
    • Monani, U.R.1    Lorson, C.L.2    Parsons, D.W.3    Prior, T.W.4    Androphy, E.J.5    Burghes, A.H.M.6
  • 15
    • 0033033434 scopus 로고    scopus 로고
    • A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophy
    • Lorson C.L., Hahnen E., Androphy E.J., Wirth B. A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophy. Proc Natl Acad Sci USA 1999, 96:6307-6311.
    • (1999) Proc Natl Acad Sci USA , vol.96 , pp. 6307-6311
    • Lorson, C.L.1    Hahnen, E.2    Androphy, E.J.3    Wirth, B.4
  • 16
    • 0037313165 scopus 로고    scopus 로고
    • Correction of disease-associated exon skipping by synthetic exon-specific activators
    • Cartegni L., Krainer A.R. Correction of disease-associated exon skipping by synthetic exon-specific activators. Nat Struct Biol 2003, 10:120-125.
    • (2003) Nat Struct Biol , vol.10 , pp. 120-125
    • Cartegni, L.1    Krainer, A.R.2
  • 17
    • 0036544654 scopus 로고    scopus 로고
    • Disruption of an SF2/ASF-dependent exonic splicing enhancer in SMN2 causes spinal muscular atrophy in the absence of SMN1
    • Cartegni L., Krainer A.R. Disruption of an SF2/ASF-dependent exonic splicing enhancer in SMN2 causes spinal muscular atrophy in the absence of SMN1. Nat Genet 2002, 30:377-384.
    • (2002) Nat Genet , vol.30 , pp. 377-384
    • Cartegni, L.1    Krainer, A.R.2
  • 18
    • 0033983258 scopus 로고    scopus 로고
    • An exonic enhancer is required for inclusion of an essential exon in the SMA-determining gene SMN
    • Lorson C.L., Androphy E.J. An exonic enhancer is required for inclusion of an essential exon in the SMA-determining gene SMN. Hum Mol Genet 2000, 9:259-265.
    • (2000) Hum Mol Genet , vol.9 , pp. 259-265
    • Lorson, C.L.1    Androphy, E.J.2
  • 19
    • 0041665176 scopus 로고    scopus 로고
    • A negative element in SMN2 exon 7 inhibits splicing in spinal muscular atrophy
    • Kashima T., Manley J.L. A negative element in SMN2 exon 7 inhibits splicing in spinal muscular atrophy. Nat Genet 2003, 34:460-463.
    • (2003) Nat Genet , vol.34 , pp. 460-463
    • Kashima, T.1    Manley, J.L.2
  • 20
    • 0035891862 scopus 로고    scopus 로고
    • Aclarubicin treatment restores SMN levels to cells derived from type I spinal muscular atrophy patients
    • Andreassi C., Jarecki J., Zhou J., Coovert D.D., Monani U.R., Chen X., et al. Aclarubicin treatment restores SMN levels to cells derived from type I spinal muscular atrophy patients. Hum Mol Genet 2001, 10:2841-2849.
    • (2001) Hum Mol Genet , vol.10 , pp. 2841-2849
    • Andreassi, C.1    Jarecki, J.2    Zhou, J.3    Coovert, D.D.4    Monani, U.R.5    Chen, X.6
  • 21
    • 0035158371 scopus 로고    scopus 로고
    • An in vivo reporter system for measuring increased inclusion of exon 7 in SMN2 mRNA: potential therapy of SMA
    • Zhang M.L., Lorson C.L., Androphy E.J., Zhou J. An in vivo reporter system for measuring increased inclusion of exon 7 in SMN2 mRNA: potential therapy of SMA. Gene Ther 2001, 8:1532-1538.
    • (2001) Gene Ther , vol.8 , pp. 1532-1538
    • Zhang, M.L.1    Lorson, C.L.2    Androphy, E.J.3    Zhou, J.4
  • 22
    • 23244458683 scopus 로고    scopus 로고
    • Hydroxyurea enhances SMN2 gene expression in spinal muscular atrophy cells
    • Grzeschik S.M., Ganta M., Prior T.W., Heavlin W.D., Wang C.H. Hydroxyurea enhances SMN2 gene expression in spinal muscular atrophy cells. Ann Neurol 2005, 58:194-202.
    • (2005) Ann Neurol , vol.58 , pp. 194-202
    • Grzeschik, S.M.1    Ganta, M.2    Prior, T.W.3    Heavlin, W.D.4    Wang, C.H.5
  • 24
    • 38349090169 scopus 로고    scopus 로고
    • Salbutamol increases SMN mRNA and protein levels in spinal muscular atrophy cells
    • Angelozzi C., Borgo F., Tiziano F.D., Martella A., Neri G., Brahe C. Salbutamol increases SMN mRNA and protein levels in spinal muscular atrophy cells. J Med Genet 2008, 45:29-31.
    • (2008) J Med Genet , vol.45 , pp. 29-31
    • Angelozzi, C.1    Borgo, F.2    Tiziano, F.D.3    Martella, A.4    Neri, G.5    Brahe, C.6
  • 26
    • 0037388256 scopus 로고    scopus 로고
    • Bifunctional antisense oligonucleotides provide a trans-acting splicing enhancer that stimulates SMN2 gene expression in patient fibroblasts
    • Skordis L.A., Dunckley M.G., Yue B., Eperon I.C., Muntoni F. Bifunctional antisense oligonucleotides provide a trans-acting splicing enhancer that stimulates SMN2 gene expression in patient fibroblasts. Proc Natl Acad Sci USA 2003, 100:4114-4119.
    • (2003) Proc Natl Acad Sci USA , vol.100 , pp. 4114-4119
    • Skordis, L.A.1    Dunckley, M.G.2    Yue, B.3    Eperon, I.C.4    Muntoni, F.5
  • 27
    • 77955894067 scopus 로고    scopus 로고
    • Antisense correction of SMN2 splicing in the CNS rescues necrosis in a type III SMA mouse model
    • Hua Y., Sahashi K., Hung G., Rigo F., Passini M.A., Bennett C.F., et al. Antisense correction of SMN2 splicing in the CNS rescues necrosis in a type III SMA mouse model. Genes Dev 2010, 24:1634-1644.
    • (2010) Genes Dev , vol.24 , pp. 1634-1644
    • Hua, Y.1    Sahashi, K.2    Hung, G.3    Rigo, F.4    Passini, M.A.5    Bennett, C.F.6
  • 28
    • 67449135902 scopus 로고    scopus 로고
    • Oligonucleotide-mediated survival of motor neuron protein expression in CNS improves phenotype in a mouse model of spinal muscular atrophy
    • Williams J.H., Schray R.C., Patterson C.A., Ayitey S.O., Tallent M.K., Lutz G.J. Oligonucleotide-mediated survival of motor neuron protein expression in CNS improves phenotype in a mouse model of spinal muscular atrophy. J Neurosci 2009, 29:7633-7638.
    • (2009) J Neurosci , vol.29 , pp. 7633-7638
    • Williams, J.H.1    Schray, R.C.2    Patterson, C.A.3    Ayitey, S.O.4    Tallent, M.K.5    Lutz, G.J.6
  • 29
    • 41549168514 scopus 로고    scopus 로고
    • Antisense masking of an hnRNP A1/A2 intronic splicing silencer corrects SMN2 splicing in transgenic mice
    • Hua Y., Vickers T.A., Okunola H.L., Bennett C.F., Krainer A.R. Antisense masking of an hnRNP A1/A2 intronic splicing silencer corrects SMN2 splicing in transgenic mice. Am J Hum Genet 2008, 82:834-848.
    • (2008) Am J Hum Genet , vol.82 , pp. 834-848
    • Hua, Y.1    Vickers, T.A.2    Okunola, H.L.3    Bennett, C.F.4    Krainer, A.R.5
  • 30
    • 77749301118 scopus 로고    scopus 로고
    • Tetracyclines that promote SMN2 exon 7 splicing as therapeutics for spinal muscular atrophy
    • 5ra12
    • Hastings M.L., Berniac J., Liu Y.H., Abato P., Jodelka F.M., Barthel L., et al. Tetracyclines that promote SMN2 exon 7 splicing as therapeutics for spinal muscular atrophy. Sci Transl Med 2009, 1. 5ra12.
    • (2009) Sci Transl Med , vol.1
    • Hastings, M.L.1    Berniac, J.2    Liu, Y.H.3    Abato, P.4    Jodelka, F.M.5    Barthel, L.6
  • 31
    • 0034327218 scopus 로고    scopus 로고
    • Interferons and IRF-1 induce expression of the survival motor neuron (SMN) genes
    • Baron-Delage S., Abadie A., Echaniz-Laguna A., Melki J., Beretta L. Interferons and IRF-1 induce expression of the survival motor neuron (SMN) genes. Mol Med 2000, 6:957-968.
    • (2000) Mol Med , vol.6 , pp. 957-968
    • Baron-Delage, S.1    Abadie, A.2    Echaniz-Laguna, A.3    Melki, J.4    Beretta, L.5
  • 33
    • 0141506887 scopus 로고    scopus 로고
    • Valproic acid increases the SMN2 protein level: a well-known drug as a potential therapy for spinal muscular atrophy
    • Brichta L., Hofmann Y., Hahnen E., Siebzehnrubl F.A., Raschke H., Blumcke I., et al. Valproic acid increases the SMN2 protein level: a well-known drug as a potential therapy for spinal muscular atrophy. Hum Mol Genet 2003, 12:2481-2489.
    • (2003) Hum Mol Genet , vol.12 , pp. 2481-2489
    • Brichta, L.1    Hofmann, Y.2    Hahnen, E.3    Siebzehnrubl, F.A.4    Raschke, H.5    Blumcke, I.6
  • 35
    • 13544258982 scopus 로고    scopus 로고
    • Phenylbutyrate increases SMN gene expression in spinal muscular atrophy patients
    • Brahe C., Vitali T., Tiziano F.D., Angelozzi C., Pinto A.M., Borgo F., et al. Phenylbutyrate increases SMN gene expression in spinal muscular atrophy patients. Eur J Hum Genet 2005, 13:256-259.
    • (2005) Eur J Hum Genet , vol.13 , pp. 256-259
    • Brahe, C.1    Vitali, T.2    Tiziano, F.D.3    Angelozzi, C.4    Pinto, A.M.5    Borgo, F.6
  • 36
    • 33745686137 scopus 로고    scopus 로고
    • The benzamide M344, a novel histone deacetylase inhibitor, significantly increases SMN2 RNA/protein levels in spinal muscular atrophy cells
    • Riessland M., Brichta L., Hahnen E., Wirth B. The benzamide M344, a novel histone deacetylase inhibitor, significantly increases SMN2 RNA/protein levels in spinal muscular atrophy cells. Hum Genet 2006, 120:101-110.
    • (2006) Hum Genet , vol.120 , pp. 101-110
    • Riessland, M.1    Brichta, L.2    Hahnen, E.3    Wirth, B.4
  • 37
    • 33745079980 scopus 로고    scopus 로고
    • In vitro and ex vivo evaluation of second-generation histone deacetylase inhibitors for the treatment of spinal muscular atrophy
    • Hahnen E., Eyüpoglu I.Y., Brichta L., Haastert K., Tränkle C., Siebzehnrübl F.A., et al. In vitro and ex vivo evaluation of second-generation histone deacetylase inhibitors for the treatment of spinal muscular atrophy. J Neurochem 2006, 98:193-202.
    • (2006) J Neurochem , vol.98 , pp. 193-202
    • Hahnen, E.1    Eyüpoglu, I.Y.2    Brichta, L.3    Haastert, K.4    Tränkle, C.5    Siebzehnrübl, F.A.6
  • 38
    • 33847358736 scopus 로고    scopus 로고
    • Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy
    • Avila A.M., Burnett B.G., Taye A.A., Gabanella F., Knight M.A., Hartenstein P., et al. Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy. J Clin Invest 2007, 117:659-671.
    • (2007) J Clin Invest , vol.117 , pp. 659-671
    • Avila, A.M.1    Burnett, B.G.2    Taye, A.A.3    Gabanella, F.4    Knight, M.A.5    Hartenstein, P.6
  • 39
    • 70350749543 scopus 로고    scopus 로고
    • LBH589 induces up to 10-fold SMN protein levels by several independent mechanisms and is effective even in cells from SMA patients non-responsive to valproate
    • Garbes L., Riessland M., Hölker I., Heller R., Hauke J., Tränkle C., et al. LBH589 induces up to 10-fold SMN protein levels by several independent mechanisms and is effective even in cells from SMA patients non-responsive to valproate. Hum Mol Genet 2009, 18:3645-3658.
    • (2009) Hum Mol Genet , vol.18 , pp. 3645-3658
    • Garbes, L.1    Riessland, M.2    Hölker, I.3    Heller, R.4    Hauke, J.5    Tränkle, C.6
  • 40
    • 33744803707 scopus 로고    scopus 로고
    • In vivo activation of SMN in spinal muscular atrophy carriers and patients treated with valproate
    • Brichta L., Holker I., Haug K., Klockgether T., Wirth B. In vivo activation of SMN in spinal muscular atrophy carriers and patients treated with valproate. Ann Neurol 2006, 59:970-975.
    • (2006) Ann Neurol , vol.59 , pp. 970-975
    • Brichta, L.1    Holker, I.2    Haug, K.3    Klockgether, T.4    Wirth, B.5
  • 41
    • 36248958610 scopus 로고    scopus 로고
    • Valproic acid treatment in six patients with spinal muscular atrophy
    • Tsai L.K., Yang C.C., Hwu W.L., Li H. Valproic acid treatment in six patients with spinal muscular atrophy. Eur J Neurol 2007, 14:e8-9.
    • (2007) Eur J Neurol , vol.14
    • Tsai, L.K.1    Yang, C.C.2    Hwu, W.L.3    Li, H.4
  • 42
    • 0021061819 scopus 로고
    • Rapid colorimetric assay for cellular growth and survival: application to proliferation and cytotoxicity assays
    • Mosmann T. Rapid colorimetric assay for cellular growth and survival: application to proliferation and cytotoxicity assays. J Immunol Methods 1983, 65:55-63.
    • (1983) J Immunol Methods , vol.65 , pp. 55-63
    • Mosmann, T.1
  • 44
    • 18244383806 scopus 로고    scopus 로고
    • Valproic acid defines a novel class of HDAC inhibitors inducing differentiation of transformed cells
    • Göttlicher M., Minucci S., Zhu P., Krämer O.H., Schimpf A., Giavara S., et al. Valproic acid defines a novel class of HDAC inhibitors inducing differentiation of transformed cells. EMBO J 2001, 20:6969-6978.
    • (2001) EMBO J , vol.20 , pp. 6969-6978
    • Göttlicher, M.1    Minucci, S.2    Zhu, P.3    Krämer, O.H.4    Schimpf, A.5    Giavara, S.6
  • 45
    • 0035965343 scopus 로고    scopus 로고
    • Histone deacetylase is a direct target of valproic acid, a potent anticonvulsant, mood stabilizer, and teratogen
    • Phiel C.J., Zhang F., Huang E.Y., Guenther M.G., Lazar M.A., Klein P.S. Histone deacetylase is a direct target of valproic acid, a potent anticonvulsant, mood stabilizer, and teratogen. J Biol Chem 2001, 276:36734-36741.
    • (2001) J Biol Chem , vol.276 , pp. 36734-36741
    • Phiel, C.J.1    Zhang, F.2    Huang, E.Y.3    Guenther, M.G.4    Lazar, M.A.5    Klein, P.S.6
  • 46
  • 47
    • 0025827397 scopus 로고
    • Comparison of valproate concentrations in human plasma, CSF and brain tissue after administration of different formulations of valproate or valpromide
    • Wieser H.G. Comparison of valproate concentrations in human plasma, CSF and brain tissue after administration of different formulations of valproate or valpromide. Epilepsy Res 1991, 9:154-159.
    • (1991) Epilepsy Res , vol.9 , pp. 154-159
    • Wieser, H.G.1
  • 48
    • 0034662922 scopus 로고    scopus 로고
    • Htra2-β1 stimulates an exonic splicing enhancer and can restore full-length SMN expression to survival motor neuron 2 (SMN2)
    • Hofmann Y., Lorson C.L., Stamm S., Androphy E.J., Wirth B. Htra2-β1 stimulates an exonic splicing enhancer and can restore full-length SMN expression to survival motor neuron 2 (SMN2). Proc Natl Acad Sci USA 2000, 97:9618-9623.
    • (2000) Proc Natl Acad Sci USA , vol.97 , pp. 9618-9623
    • Hofmann, Y.1    Lorson, C.L.2    Stamm, S.3    Androphy, E.J.4    Wirth, B.5
  • 49
    • 0037101846 scopus 로고    scopus 로고
    • HnRNP-G promotes exon 7 inclusion of survival motor neuron (SMN) via direct interaction with Htra2-β1
    • Hofmann Y., Wirth B. HnRNP-G promotes exon 7 inclusion of survival motor neuron (SMN) via direct interaction with Htra2-β1. Hum Mol Genet 2002, 11:2037-2049.
    • (2002) Hum Mol Genet , vol.11 , pp. 2037-2049
    • Hofmann, Y.1    Wirth, B.2
  • 50
    • 35148878581 scopus 로고    scopus 로고
    • Histone deacetylase inhibitors reduce steroidogenesis through SCF-mediated ubiquitination and degradation of steroidogenic factor 1 (NR5A1)
    • Chen W.Y., Weng J.H., Huang C.C., Chung B.C. Histone deacetylase inhibitors reduce steroidogenesis through SCF-mediated ubiquitination and degradation of steroidogenic factor 1 (NR5A1). Mol Cell Biol 2007, 27:7284-7290.
    • (2007) Mol Cell Biol , vol.27 , pp. 7284-7290
    • Chen, W.Y.1    Weng, J.H.2    Huang, C.C.3    Chung, B.C.4
  • 51
    • 0037119437 scopus 로고    scopus 로고
    • Distinct sets of adjacent heterogeneous nuclear ribonucleoprotein (hnRNP) A1/A2 binding sites control 5' splice site selection in the hnRNP A1 mRNA precursor
    • Hutchison S., LeBel C., Blanchette M., Chabot B. Distinct sets of adjacent heterogeneous nuclear ribonucleoprotein (hnRNP) A1/A2 binding sites control 5' splice site selection in the hnRNP A1 mRNA precursor. J Biol Chem 2002, 277:29745-29752.
    • (2002) J Biol Chem , vol.277 , pp. 29745-29752
    • Hutchison, S.1    LeBel, C.2    Blanchette, M.3    Chabot, B.4
  • 52
    • 33747083488 scopus 로고    scopus 로고
    • Valproate may improve strength and function in patients with type III/IV spinal muscle atrophy
    • Weihl C.C., Connolly A.M., Pestronk A. Valproate may improve strength and function in patients with type III/IV spinal muscle atrophy. Neurology 2006, 67:500-501.
    • (2006) Neurology , vol.67 , pp. 500-501
    • Weihl, C.C.1    Connolly, A.M.2    Pestronk, A.3
  • 53
    • 52149098852 scopus 로고    scopus 로고
    • Hand-held dynamometry: reliability of lower extremity muscle testing in healthy, physically active, young adults
    • Kelln B.M., McKeon P.O., Gontkof L.M., Hertel J. Hand-held dynamometry: reliability of lower extremity muscle testing in healthy, physically active, young adults. J Sport Rehabil 2008, 17:160-170.
    • (2008) J Sport Rehabil , vol.17 , pp. 160-170
    • Kelln, B.M.1    McKeon, P.O.2    Gontkof, L.M.3    Hertel, J.4
  • 56
    • 0027223365 scopus 로고
    • Valproate and sodium currents in cultured hippocampal neurons
    • Van den Berg R.J., Kok P., Voskuyl R.A. Valproate and sodium currents in cultured hippocampal neurons. Exp Brain Res 1993, 93:279-287.
    • (1993) Exp Brain Res , vol.93 , pp. 279-287
    • Van den Berg, R.J.1    Kok, P.2    Voskuyl, R.A.3
  • 57
    • 77957929588 scopus 로고    scopus 로고
    • S.M.A.C.A.R.N.I.-V.A.L. trial part I: double-blind, randomized, placebo-controlled trial of L-carnitine and valproic acid in spinal muscular atrophy
    • Swoboda K.J., Scott C.B., Crawford T.O., Simard L.R., Reyna S.P., Krosschell K.J., et al. S.M.A.C.A.R.N.I.-V.A.L. trial part I: double-blind, randomized, placebo-controlled trial of L-carnitine and valproic acid in spinal muscular atrophy. PLoS One 2010, 5:e12140.
    • (2010) PLoS One , vol.5
    • Swoboda, K.J.1    Scott, C.B.2    Crawford, T.O.3    Simard, L.R.4    Reyna, S.P.5    Krosschell, K.J.6


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.