MeCP2 in Rett syndrome: transcriptional repressor or chromatin architectural protein?

被引:34
作者
Chadwick, Lisa Helbling [1 ]
Wade, Paul A. [1 ]
机构
[1] Natl Inst Environm Hlth Sci, Lab Mol Carcinogenesis, Res Triangle Pk, NC 27709 USA
关键词
D O I
10.1016/j.gde.2007.02.003
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
摘要
Rett syndrome is a progressive neurological disorder caused by mutations in the methyl-DNA binding protein MeCP2. The longstanding model depicting MeCP2 as a transcriptional repressor predicts that the Rett syndrome phenotype probably results from misregulation of MeCP2 target genes. Somewhat unexpectedly, the identification of such targets has proven challenging. The recent identification of two MeCP2 targets, BDNF and DLX5, are suggestive of two very different roles for this protein - one as a classical repressor protein, and the other as a mediator of a complex, specialized chromatin structure.
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收藏
页码:121 / 125
页数:5
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共 38 条
  • [1] Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2
    Amir, RE
    Van den Veyver, IB
    Wan, M
    Tran, CQ
    Francke, U
    Zoghbi, HY
    [J]. NATURE GENETICS, 1999, 23 (02) : 185 - 188
  • [2] The impact of MECP2 mutations in the expression patterns of Rett syndrome patients
    Ballestar, E
    Ropero, S
    Alaminos, M
    Armstrong, J
    Setien, F
    Agrelo, R
    Fraga, MF
    Herranz, M
    Avila, S
    Pineda, M
    Monros, E
    Esteller, M
    [J]. HUMAN GENETICS, 2005, 116 (1-2) : 91 - 104
  • [3] MECP2 mutations in Rett syndrome adversely affect lymphocyte growth, but do not affect imprinted gene expression in blood or brain
    Balmer, D
    Arredondo, J
    Samaco, RC
    LaSalle, JM
    [J]. HUMAN GENETICS, 2002, 110 (06) : 545 - 552
  • [4] The disease progression mutant mice is affected of Mecp2 by the level of BDNF expression
    Chang, QA
    Khare, G
    Dani, V
    Nelson, S
    Jaenisch, R
    [J]. NEURON, 2006, 49 (03) : 341 - 348
  • [5] Derepression of BDNF transcription involves calcium-dependent phosphorylation of MeCP2
    Chen, WG
    Chang, Q
    Lin, YX
    Meissner, A
    West, AE
    Griffith, EC
    Jaenisch, R
    Greenberg, ME
    [J]. SCIENCE, 2003, 302 (5646) : 885 - 889
  • [6] Chen WG, 2003, J NEUROSCI, V23, P2572
  • [7] Gene expression profiling in postmortem Rett syndrome brain: Differential gene expression and patient classification
    Colantuoni, C
    Jeon, OH
    Hyder, K
    Chenchik, A
    Khimani, AH
    Narayanan, V
    Hoffman, EP
    Kaufmann, WE
    Naidu, S
    Pevsner, J
    [J]. NEUROBIOLOGY OF DISEASE, 2001, 8 (05) : 847 - 865
  • [8] Reduced cortical activity due to a shift in the balance between excitation and inhibition in a mouse model of Rett Syndrome
    Dani, VS
    Chang, Q
    Maffei, A
    Turrigiano, GG
    Jaenisch, R
    Nelson, SB
    [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2005, 102 (35) : 12560 - 12565
  • [9] A 5' differentially methylated sequence and the 3'-flanking region are necessary for H19 transgene imprinting
    Elson, DA
    Bartolomei, MS
    [J]. MOLECULAR AND CELLULAR BIOLOGY, 1997, 17 (01) : 309 - 317
  • [10] Chromatin compaction by a polycomb group protein complex
    Francis, NJ
    Kingston, RE
    Woodcock, CL
    [J]. SCIENCE, 2004, 306 (5701) : 1574 - 1577