X inactivation and disease

被引:15
作者
Agrelo, Ruben [1 ]
Wutz, Anton [1 ]
机构
[1] Res Inst Mol Pathol, A-1030 Vienna, Austria
关键词
X inactivation; Xist RNA; Chromatin; Dosage compensation; Stem cells; Gene regulation; Epigenetics; XIST RNA; CHROMOSOME INACTIVATION; DNA METHYLATION; MOUSE; EXPRESSION; GENE; CHROMATIN; PROTEIN; LOCALIZATION; LYMPHOMA;
D O I
10.1016/j.semcdb.2009.09.017
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
摘要
X inactivation is the mechanism by which mammals adjust the X-linked gene dosage between the sexes. The dosage difference between XX females and XY males is functionally equalized by silencing one of the two X chromosomes in female cells. This dosage-compensation mechanism is based on the long functional Xist RNA. Here, we review our understanding of dosage compensation and Xist function in the context of disease. (C) 2009 Elsevier Ltd. All rights reserved.
引用
收藏
页码:194 / 200
页数:7
相关论文
共 69 条
[1]   SATB1 Defines the Developmental Context for Gene Silencing by Xist in Lymphoma and Embryonic Cells [J].
Agrelo, Ruben ;
Souabni, Abdallah ;
Novatchkova, Maria ;
Haslinger, Christian ;
Leeb, Martin ;
Komnenovic, Vukoslav ;
Kishimoto, Hiroyuki ;
Gresh, Lionel ;
Kohwi-Shigematsu, Terumi ;
Kenner, Lukas ;
Wutz, Anton .
DEVELOPMENTAL CELL, 2009, 16 (04) :507-516
[2]   X-chromosome kiss and tell: How the Xs go their separate ways [J].
Anguera, M. C. ;
Sun, B. K. ;
Xu, N. ;
Lee, J. T. .
COLD SPRING HARBOR SYMPOSIA ON QUANTITATIVE BIOLOGY, 2006, 71 :429-437
[3]  
Bacher CP, 2006, NAT CELL BIOL, V8, P293, DOI 10.1038/ncb1365
[4]   Mbd2 contributes to DNA methylation-directed repression of the Xist gene [J].
Barr, Helen ;
Hermann, Andrea ;
Berger, Jennifer ;
Tsai, Hsin-Hao ;
Adie, Karen ;
Prokhortchouk, Anna ;
Hendrich, Brian ;
Bird, Adrian .
MOLECULAR AND CELLULAR BIOLOGY, 2007, 27 (10) :3750-3757
[5]   LOSS OF METHYLATION ACTIVATES XIST IN SOMATIC BUT NOT IN EMBRYONIC-CELLS [J].
BEARD, C ;
LI, E ;
JAENISCH, R .
GENES & DEVELOPMENT, 1995, 9 (19) :2325-2334
[6]   SmcHD1, containing a structural-maintenance-of-chromosomes hinge domain, has a critical role in X inactivation [J].
Blewitt, Marnie E. ;
Gendrel, Anne-Valerie ;
Pang, Zhenyi ;
Sparrow, Duncan B. ;
Whitelaw, Nadia ;
Craig, Jeffrey M. ;
Apedaile, Anwyn ;
Hilton, Douglas J. ;
Dunwoodie, Sally L. ;
Brockdorff, Neil ;
Kay, Graham F. ;
Whitelaw, Emma .
NATURE GENETICS, 2008, 40 (05) :663-669
[7]   Histone modification patterns associated with the human X chromosome [J].
Brinkman, Arie B. ;
Roelofsen, Thijs ;
Pennings, Sebastiaan W. C. ;
Martens, Joost H. A. ;
Jenuwein, Thomas ;
Stunnenberg, Hendrik G. .
EMBO REPORTS, 2006, 7 (06) :628-634
[8]   SAT in Silence [J].
Brockdorff, Neil .
DEVELOPMENTAL CELL, 2009, 16 (04) :483-484
[9]   SATB1 packages densely looped, transcriptionally active chromatin for coordinated expression of cytokine genes [J].
Cai, Shutao ;
Lee, Charles C. ;
Kohwi-Shigematsu, Terumi .
NATURE GENETICS, 2006, 38 (11) :1278-1288
[10]   A novel role for Xist RNA in the formation of a repressive nuclear compartment into which genes are recruited when silenced [J].
Chaumeil, Julie ;
Le Baccon, Patricia ;
Wutz, Anton ;
Heard, Edith .
GENES & DEVELOPMENT, 2006, 20 (16) :2223-2237