Increased Steady-State Levels of CUGBP1 in Myotonic Dystrophy 1 Are Due to PKC-Mediated Hyperphosphorylation

Molecular Cell - Tập 28 - Trang 68-78 - 2007
N. Muge Kuyumcu-Martinez1, Guey-Shin Wang1, Thomas A. Cooper1,2
1Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA
2Department of Molecular and Cellular Biology, Baylor College of Medicine, Houston, TX 77030, USA

Tài liệu tham khảo

Cardani, 2006, Muscleblind-like protein 1 nuclear sequestration is a molecular pathology marker of DM1 and DM2, Eur. J. Histochem., 50, 177 Carnac, 1998, RhoA GTPase and serum response factor control selectively the expression of MyoD without affecting Myf5 in mouse myoblasts, Mol. Biol. Cell, 9, 1891, 10.1091/mbc.9.7.1891 Charlet-B, 2002, Loss of the muscle-specific chloride channel in type 1 myotonic dystrophy due to misregulated alternative splicing, Mol. Cell, 10, 45, 10.1016/S1097-2765(02)00572-5 Dansithong, 2005, MBNL1 is the primary determinant of focus formation and aberrant insulin receptor splicing in DM1, J. Biol. Chem., 280, 5773, 10.1074/jbc.M410781200 Fardaei, 2001, In vivo co-localisation of MBNL protein with DMPK expanded-repeat transcripts, Nucleic Acids Res., 29, 2766, 10.1093/nar/29.13.2766 Fardaei, 2002, Three proteins, MBNL, MBLL and MBXL, co-localize in vivo with nuclear foci of expanded-repeat transcripts in DM1 and DM2 cells, Hum. Mol. Genet., 11, 805, 10.1093/hmg/11.7.805 Fields, 1988, Phosphorylation of lamin B at the nuclear membrane by activated protein kinase C, J. Biol. Chem., 263, 8253, 10.1016/S0021-9258(18)68471-6 Gysin, 1996, Replacement of Ser657 of protein kinase C-α by alanine leads to premature down regulation after phorbol-ester-induced translocation to the membrane, Eur. J. Biochem., 240, 747, 10.1111/j.1432-1033.1996.0747h.x Hamplova, 2005, Protein kinase C activity and isoform expression during early postnatal development of rat myocardium, Cell Biochem. Biophys., 43, 105, 10.1385/CBB:43:1:105 Harper, 2001, Volume 37 Ho, 2004, Muscleblind proteins regulate alternative splicing, EMBO J., 23, 3103, 10.1038/sj.emboj.7600300 Ho, 2005, Transgenic mice expressing CUG-BP1 reproduce splicing mis-regulation observed in myotonic dystrophy, Hum. Mol. Genet., 14, 1539, 10.1093/hmg/ddi162 Ho, 2005, Co-localization of muscleblind with RNA foci is separable from mis-regulation of alternative splicing in myotonic dystrophy, J. Cell Sci., 118, 2923, 10.1242/jcs.02404 Jiang, 2004, Myotonic dystrophy type 1 is associated with nuclear foci of mutant RNA, sequestration of muscleblind proteins and deregulated alternative splicing in neurons, Hum. Mol. Genet., 13, 3079, 10.1093/hmg/ddh327 Kanadia, 2003, A muscleblind knockout model for myotonic dystrophy, Science, 302, 1978, 10.1126/science.1088583 Kuyumcu-Martinez, 2006, Misregulation of alternative splicing causes pathogenesis in myotonic dystrophy, Prog. Mol. Subcell. Biol., 44, 133, 10.1007/978-3-540-34449-0_7 Ladd, 2005, Dynamic balance between activation and repression regulates pre-mRNA alternative splicing during heart development, Dev. Dyn., 233, 783, 10.1002/dvdy.20382 Lin, 2006, Failure of MBNL1-dependent post-natal splicing transitions in myotonic dystrophy, Hum. Mol. Genet., 15, 2087, 10.1093/hmg/ddl132 Lueck, 2007, Chloride channelopathy in myotonic dystrophy resulting from loss of post-transcriptional regulation for CLCN1, Am. J. Physiol. Cell Physiol., 292, C1245, 10.1152/ajpcell.00336.2006 Mahadevan, 2006, Reversible model of RNA toxicity and cardiac conduction defects in myotonic dystrophy, Nat. Genet., 38, 1066, 10.1038/ng1857 Mankodi, 2002, Expanded CUG repeats trigger aberrant splicing of ClC-1 chloride channel pre-mRNA and hyperexcitability of skeletal muscle in myotonic dystrophy, Mol. Cell, 10, 35, 10.1016/S1097-2765(02)00563-4 Mankodi, 2005, Nuclear RNA foci in the heart in myotonic dystrophy, Circ. Res., 97, 1152, 10.1161/01.RES.0000193598.89753.e3 Martelli, 2003, Nuclear protein kinase C isoforms: key players in multiple cell functions?, Histol. Histopathol., 18, 1301 Martelli, 2006, Nuclear protein kinase C, Biochim. Biophys. Acta, 1761, 542, 10.1016/j.bbalip.2006.02.009 Musashi, 2000, The role of protein kinase C isoforms in cell proliferation and apoptosis, Int. J. Hematol., 72, 12 Ng, 2001, Ezrin is a donwstream effector of trafficking PKC-integrin complexes involved in the control of cell motility, EMBO J., 20, 2723, 10.1093/emboj/20.11.2723 Omri, 1987, Characteristics of thyroid protein kinase C. Different Ca2 requirement for the phosphorylation of endogenous proteins and of H1 histone, Eur. J. Biochem., 165, 83, 10.1111/j.1432-1033.1987.tb11197.x Pascale, 2005, Neuronal ELAV proteins enhance mRNA stability by a PKCα-dependent pathway, Proc. Natl. Acad. Sci. USA, 102, 12065, 10.1073/pnas.0504702102 Philips, 1998, Disruption of splicing regulated by a CUG-binding protein in myotonic dystrophy, Science, 280, 737, 10.1126/science.280.5364.737 Ranum, 2006, RNA-mediated neuromuscular disorders, Annu. Rev. Neurosci., 38, 758 Roberts, 1997, Altered phosphorylation and intracellular distribution of a (CUG)n triplet repeat RNA-binding protein in patients with myotonic dystrophy and in myotonin protein kinase knockout mice, Proc. Natl. Acad. Sci. USA, 94, 13221, 10.1073/pnas.94.24.13221 Savkur, 2001, Aberrant regulation of insulin receptor alternative splicing is associated with insulin resistance in myotonic dystrophy, Nat. Genet., 29, 40, 10.1038/ng704 Schreiber, 2001, Protein kinase C isoform expression and activity in the mouse heart, Am. J. Physiol. Heart Circ. Physiol., 281, H2062, 10.1152/ajpheart.2001.281.5.H2062 Timchenko, 1996, Novel proteins with binding specificity for DNA CTG repeats and RNA CUG repeats: implications for myotonic dystrophy, Hum. Mol. Genet., 5, 115, 10.1093/hmg/5.1.115 Timchenko, 2001, RNA CUG repeats sequester CUGBP1 and alter protein levels and activity of CUGBP1, J. Biol. Chem., 276, 7820, 10.1074/jbc.M005960200 Timchenko, 2004, Overexpression of CUG triplet repeat-binding protein, CUGBP1, in mice inhibits myogenesis, J. Biol. Chem., 279, 13129, 10.1074/jbc.M312923200 Vijayan, 2004, Protein kinase C-α-induced hypertrophy of neonatal rat ventricular myocytes, Am. J. Physiol. Heart Circ. Physiol., 287, H2777, 10.1152/ajpheart.00171.2004 Wagner, 2000, Analysis of the subcellular distribution of protein kinase Cα using PKC-GFP fusion proteins, Exp. Cell Res., 258, 204, 10.1006/excr.2000.4925 Wang, 2007, Elevated CUGBP1 is an early event in an inducible heart-specific mouse model of myotonic dystrophy, J. Clin. Invest., 10.1172/JCI32308 Weintraub, 1989, Activation of muscle-specific genes in pigment, nerve, fat, liver, and fibroblast cell lines by forced expression of MyoD, Proc. Natl. Acad. Sci. USA, 86, 5434, 10.1073/pnas.86.14.5434