A-type lamins: Guardians of the soma?

被引：170
作者：
Hutchison, CJ
Worman, HJ
机构：
[1] Univ Durham, Sch Biol & Biomed Sci, Durham DH1 4EB, England
[2] Columbia Univ, Coll Phys & Surg, New York, NY 10032 USA
来源：
NATURE CELL BIOLOGY | 2004年 / 6卷 / 11期
基金：
美国国家卫生研究院; 英国惠康基金;
关键词：
D O I：
10.1038/ncb1104-1062
中图分类号：
Q2 [细胞生物学];
学科分类号：
071009 ; 090102 ;
摘要：
The gene LMNA encodes the proteins lamins A and C and is implicated in nine different laminopathies-inherited diseases that are linked to premature ageing. Recent evidence has demonstrated that lamins A and C have essential functions in protecting cells from physical damage, as well as in maintaining the function of transcription factors required for the differentiation of adult stem cells. Thus, the degenerative nature of laminopathies is explained because these lamins are essential for maintenance of somatic tissues in adulthood.
引用
收藏
页码：1062 / 1067
页数：6
相关论文
共 95 条
  • [1] THE NUCLEAR LAMINA IS A MESHWORK OF INTERMEDIATE-TYPE FILAMENTS
    AEBI, U
    COHN, J
    BUHLE, L
    GERACE, L
    [J]. NATURE, 1986, 323 (6088) : 560 - 564
  • [2] Zinc metalloproteinase, ZMPSTE24, is mutated in mandibuloacral dysplasia
    Agarwal, AK
    Fryns, JP
    Auchus, RJ
    Garg, A
    [J]. HUMAN MOLECULAR GENETICS, 2003, 12 (16) : 1995 - 2001
  • [3] Syne-1, a dystrophin- and klarsicht-related protein associated with synaptic nuclei at the neuromuscular junction
    Apel, ED
    Lewis, RM
    Grady, RM
    Sanes, JR
    [J]. JOURNAL OF BIOLOGICAL CHEMISTRY, 2000, 275 (41) : 31986 - 31995
  • [4] Multiple and surprising new functions for emerin, a nuclear membrane protein
    Bengtsson, L
    Wilson, KL
    [J]. CURRENT OPINION IN CELL BIOLOGY, 2004, 16 (01) : 73 - 79
  • [5] Zmpste24 deficiency in mice causes spontaneous bone fractures, muscle weakness, and a prelamin A processing defect
    Bergo, MO
    Gavino, B
    Ross, J
    Schmidt, WK
    Hong, C
    Kendall, LV
    Mohr, A
    Meta, M
    Genant, H
    Jiang, YB
    Wisner, ER
    van Bruggen, N
    Carano, RAD
    Michaelis, S
    Griffey, SM
    Young, SG
    [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2002, 99 (20) : 13049 - 13054
  • [6] IDENTIFICATION OF A NOVEL X-LINKED GENE RESPONSIBLE FOR EMERY-DREIFUSS MUSCULAR-DYSTROPHY
    BIONE, S
    MAESTRINI, E
    RIVELLA, S
    MANCINI, M
    REGIS, S
    ROMEO, G
    TONIOLO, D
    [J]. NATURE GENETICS, 1994, 8 (04) : 323 - 327
  • [7] Bonne G, 2000, ANN NEUROL, V48, P170, DOI 10.1002/1531-8249(200008)48:2<170::AID-ANA6>3.0.CO
  • [8] 2-J
  • [9] Mutations in the gene encoding lamin A/C cause autosomal dominant Emery-Dreifuss muscular dystrophy
    Bonne, G
    Di Barletta, MR
    Varnous, S
    Bécane, HM
    Hammouda, EH
    Merlini, L
    Muntoni, F
    Greenberg, CR
    Gary, F
    Urtizberea, JA
    Duboc, D
    Fardeau, M
    Toniolo, D
    Schwartz, K
    [J]. NATURE GENETICS, 1999, 21 (03) : 285 - 288
  • [10] Aging of Hutchinson-Gilford progeria syndrome fibroblasts is characterised by hyperproliferation and increased apoptosis
    Bridger, JM
    Kill, IR
    [J]. EXPERIMENTAL GERONTOLOGY, 2004, 39 (05) : 717 - 724
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