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Volumn 175, Issue 2, 2009, Pages 748-762

Expression of RNA CCUG repeats dysregulates translation and degradation of proteins in myotonic dystrophy 2 patients

Author keywords

[No Author keywords available]

Indexed keywords

CUG TRIPLET REPEAT RNA BINDING PROTEIN 1; CYTOPLASM PROTEIN; INITIATION FACTOR; MULTIPROTEIN COMPLEX; PROTEASOME; RNA; RNA BINDING PROTEIN; TRANSLATION INITIATION FACTOR EIF2; UNCLASSIFIED DRUG;

EID: 67650751240     PISSN: 00029440     EISSN: 15252191     Source Type: Journal    
DOI: 10.2353/ajpath.2009.090047     Document Type: Article
Times cited : (69)

References (29)
  • 2
    • 2342461060 scopus 로고    scopus 로고
    • Myotonic dystrophy: RNA pathogenesis comes into focus
    • Ranum LP, Day JW: Myotonic dystrophy: RNA pathogenesis comes into focus. Am J Hum Genet 2004, 74:793-804
    • (2004) Am J Hum Genet , vol.74 , pp. 793-804
    • Ranum, L.P.1    Day, J.W.2
  • 10
    • 0030043709 scopus 로고    scopus 로고
    • Novel proteins with binding specificity to DNA CTG and RNA CUG repeats: Implications for myotonic dystrophy
    • Timchenko LT, Timchenko NA, Caskey CT, Roberts R: Novel proteins with binding specificity to DNA CTG and RNA CUG repeats: implications for myotonic dystrophy. Hum Mol Genet 1996, 5:115-121
    • (1996) Hum Mol Genet , vol.5 , pp. 115-121
    • Timchenko, L.T.1    Timchenko, N.A.2    Caskey, C.T.3    Roberts, R.4
  • 13
    • 1842529234 scopus 로고    scopus 로고
    • Overexpression of CUG triplet repeat-binding protein. CUGBP1, in mice inhibits myogenesis
    • Timchenko NA, Patel R, Iakova P, Cai Z-J, Quan L, Timchenko LT: Overexpression of CUG triplet repeat-binding protein. CUGBP1, in mice inhibits myogenesis J Biol Chem 2004, 279:13129-13139
    • (2004) J Biol Chem , vol.279 , pp. 13129-13139
    • Timchenko, N.A.1    Patel, R.2    Iakova, P.3    Cai, Z.-J.4    Quan, L.5    Timchenko, L.T.6
  • 14
    • 20444452898 scopus 로고    scopus 로고
    • Transgenic mice expressing CUG-BP1 reproduce splicing mis-regulation observed in myotonic dystrophy
    • Ho THH, Bundman D, Armstong DL, Cooper TA: Transgenic mice expressing CUG-BP1 reproduce splicing mis-regulation observed in myotonic dystrophy. Hum Mol Genet 2005, 14:1539-1547
    • (2005) Hum Mol Genet , vol.14 , pp. 1539-1547
    • Ho, T.H.H.1    Bundman, D.2    Armstong, D.L.3    Cooper, T.A.4
  • 19
    • 0032076126 scopus 로고    scopus 로고
    • Disruption of splicing regulated by CUG-binding protein in myotonic dystrophy
    • Philips AV, Timchenko LT, Cooper TA: Disruption of splicing regulated by CUG-binding protein in myotonic dystrophy. Science 1998, 280:737-741
    • (1998) Science , vol.280 , pp. 737-741
    • Philips, A.V.1    Timchenko, L.T.2    Cooper, T.A.3
  • 20
    • 0036347927 scopus 로고    scopus 로고
    • Loss of the muscle-specific chloride channel in type 1 myotonic dystrophy due to misregulated alternative splicing
    • Charlet-BN, Savcur RS, Singh G, Philips AV, Grice EA, Cooper TA: Loss of the muscle-specific chloride channel in type 1 myotonic dystrophy due to misregulated alternative splicing. Mol Cell 2002, 10:43-45
    • (2002) Mol Cell , vol.10 , pp. 43-45
    • BN, C.1    Savcur, R.S.2    Singh, G.3    Philips, A.V.4    Grice, E.A.5    Cooper, T.A.6
  • 21
    • 33744980908 scopus 로고    scopus 로고
    • Mammalian CELF/ Bruno-like RNA-binding proteins: Molecular characteristics and biological functions
    • Barreau C, Paillard L, Mereau A, Osborne BH: Mammalian CELF/ Bruno-like RNA-binding proteins: molecular characteristics and biological functions. Biochimie 2005, 88:515-525
    • (2005) Biochimie , vol.88 , pp. 515-525
    • Barreau, C.1    Paillard, L.2    Mereau, A.3    Osborne, B.H.4
  • 23
    • 0029746150 scopus 로고    scopus 로고
    • In vitro synthesis and cloning of long CAG repeats
    • Ordway JM, Detloff PJ: In vitro synthesis and cloning of long CAG repeats. BioTechniques 1996, 21:609-610
    • (1996) BioTechniques , vol.21 , pp. 609-610
    • Ordway, J.M.1    Detloff, P.J.2
  • 24
    • 0037040541 scopus 로고    scopus 로고
    • Molecular chaperones in the cytosol: From nascent chain to folded protein
    • Hartl FU, Hayer-Hartl M: Molecular chaperones in the cytosol: from nascent chain to folded protein. Science 2002, 295:1852-1858
    • (2002) Science , vol.295 , pp. 1852-1858
    • Hartl, F.U.1    Hayer-Hartl, M.2
  • 25
    • 0346727127 scopus 로고    scopus 로고
    • Protein degradation and protection against misfolded or damaged proteins
    • Goldberg AL: Protein degradation and protection against misfolded or damaged proteins. Nature 2003, 426:895-899
    • (2003) Nature , vol.426 , pp. 895-899
    • Goldberg, A.L.1
  • 26
    • 0033869876 scopus 로고    scopus 로고
    • Translational control of C/EBPα and C/EBPβ isoform expression
    • Calkhoven CF, Muller C, Leutz A: Translational control of C/EBPα and C/EBPβ isoform expression. Genes Dev 2000, 14:1920-1932
    • (2000) Genes Dev , vol.14 , pp. 1920-1932
    • Calkhoven, C.F.1    Muller, C.2    Leutz, A.3
  • 27
    • 25844487226 scopus 로고    scopus 로고
    • Diseases of unstable repeat expansions: Mechanisms and common principles
    • Gatchel JR, Zoghbi HY: Diseases of unstable repeat expansions: mechanisms and common principles. Nat Rev Genet 2005, 10:743-755
    • (2005) Nat Rev Genet , vol.10 , pp. 743-755
    • Gatchel, J.R.1    Zoghbi, H.Y.2
  • 29
    • 33744762160 scopus 로고    scopus 로고
    • DM2 intronic expansions: Evidence for CCUG accumulation without flanking sequence or effects on ZNF9 mRNA processing or protein expression
    • Margolis JM, Schoser BG, Moseley ML, Day JW, Ranum LPW: DM2 intronic expansions: evidence for CCUG accumulation without flanking sequence or effects on ZNF9 mRNA processing or protein expression. Hum Mol Genet 2006, 15:1808-1815
    • (2006) Hum Mol Genet , vol.15 , pp. 1808-1815
    • Margolis, J.M.1    Schoser, B.G.2    Moseley, M.L.3    Day, J.W.4    Ranum, L.P.W.5


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.