國家衛生研究院 NHRI:Item 3990099045/8502
English  |  正體中文  |  简体中文  |  全文笔数/总笔数 : 12145/12927 (94%)
造访人次 : 910509      在线人数 : 829
RC Version 6.0 © Powered By DSPACE, MIT. Enhanced by NTU Library IR team.
搜寻范围 查询小技巧：
  • 您可在西文检索词汇前后加上"双引号"，以获取较精准的检索结果
  • 若欲以作者姓名搜寻，建议至进阶搜寻限定作者字段，可获得较完整数据
    •  进阶搜寻
    主页 ‧ 登入 ‧ 上传 ‧ 说明 ‧ 关于NHRI ‧ 管理 到手机版


    jsp.display-item.identifier=請使用永久網址來引用或連結此文件: http://ir.nhri.org.tw/handle/3990099045/8502


    题名: Dysregulated affinity of lamin A to SUN1 induces nuclear and endoplasmic reticulum aberrancies in progeric laminopathies
    作者: Chi, YH
    贡献者: Institute of Biotechnology and Pharmaceutical Research
    摘要: Hutchinson-Gilford progeria syndrome (HGPS) is a human progeroid disease caused by a point mutation on the LMNA gene. We have previously reported that the accumulation of the nuclear envelope (NE) protein, SUN1, contributes to HGPS nuclear aberrancies. However, how the interaction between the mutant lamin A (also known as progerin or LAΔ50) and SUN1 produces HGPS cellular phenotypes requires further elucidation. Using light and electron microscopy, this study demonstrated that SUN1 contributes to progerin-elicited structural changes in the NE and the endoplasmic reticulum (ER) network. This study identified the full-length lamin A associated with SUN1 in two domains, with the farnesylated cysteine within the CaaX motif (C661farnesyl) showing a stronger affinity to SUN1 than to the amino acids within 607 to 656 (LMNA-SID). Farnesylation of progerin enhances its interaction with SUN1 and reduces SUN1 mobility, thereby promoting the aberrant recruitment of the ER membrane during postmitotic assembly of the nuclear envelope, and resulting in the accumulation of SUN1 over consecutive cellular divisions. These results indicate that the dysregulated interaction of SUN1 and progerin in the ER during NE reformation determines the progression of HGPS.
    日期: 2014-04
    關聯: FASEB Journal. 2014 Apr;28(1 Suppl.):Article number 1009.1.
    Link to: http://www.fasebj.org/content/28/1_Supplement/1009.1.abstract?sid=9510b0cd-68c8-4d1f-950e-446519d14952
    JIF/Ranking 2023: http://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcAuth=NHRI&SrcApp=NHRI_IR&KeyISSN=0892-6638&DestApp=IC2JCR
    Cited Times(WOS): https://www.webofscience.com/wos/woscc/full-record/WOS:000346651000045
    显示于类别:[紀雅惠] 會議論文/會議摘要

    文件中的档案:

    没有与此文件相关的档案.



    在NHRI中所有的数据项都受到原著作权保护.

    TAIR相关文章

    DSpace Software Copyright © 2002-2004  MIT &  Hewlett-Packard  /   Enhanced by   NTU Library IR team Copyright ©   - 回馈