北京大学医学部机构知识库
Advanced  
IR@PKUHSC  > 北京大学第一临床医学院  > 儿科  > 期刊论文
学科主题: 临床医学
题名:
R168H and V165X mutant podocin might induce different degrees of podocyte injury via different molecular mechanisms
作者: Fan, Qingfeng1; Zhang, Han1; Ding, Jie1; Liu, Shufang1; Miao, Jing1; Xing, Yan2; Yu, Zihua3; Guan, Na1
刊名: GENES TO CELLS
发表日期: 2009-09-01
DOI: 10.1111/j.1365-2443.2009.01336.x
卷: 14, 期:9, 页:1079-1090
收录类别: SCI
文章类型: Article
WOS标题词: Science & Technology
类目[WOS]: Cell Biology ; Genetics & Heredity
研究领域[WOS]: Cell Biology ; Genetics & Heredity
关键词[WOS]: FOCAL SEGMENTAL GLOMERULOSCLEROSIS ; ENDOPLASMIC-RETICULUM STRESS ; GLOMERULAR SLIT DIAPHRAGM ; EPITHELIAL-CELL INJURY ; NEPHROTIC SYNDROME ; PLASMA-MEMBRANE ; NPHS2 ; TRPC6 ; CHANNEL ; KIDNEY
英文摘要:

A lot of mutations of podocin, a key protein of podocyte slit diaphragm (SD), have been found both in hereditary and sporadic focal segmental glomeruloscleorosis (FSGS). Nevertheless, the mechanisms of podocyte injury induced by mutant podocins are still unclear. A compound heterozygous podocin mutation was identified in our FSGS patient, leading to a truncated (podocin V165X) and a missense mutant protein (podocin R168H), respectively. Here, it was explored whether and how both mutant podocins induce podocyte injury in the in vitro cultured podocyte cell line. Our results showed that podocin R168H induced more significant podocyte apoptosis and expression changes in more podocyte molecules than podocin V165X. Podocyte injury caused by the normal localized podocin V165X was effectively inhibited by TRPC6 knockdown. The abnormal retention of podocin R168H in endoplasmic reticulum ( ER) resulted in the mis-localizations of other critical SD molecules nephrin, CD2AP and TRPC6, and significantly up-regulated ER stress markers Bip/grp78, p-PERK and caspase-12. These results implicated that podocin R168H and podocin V165X induced different degrees of podocyte injury, which might be resulted from different molecular mechanisms. Our findings provided some possible clues for further exploring the pharmacological targets to the proteinuria induced by different mutant podocins.

语种: 英语
所属项目编号: 30170992 ; 30672259 ; 30801250 ; 7072080 ; 20070001764
项目资助者: National Nature Science Foundation of China ; Beijing Nature Science Foundation ; Chinese Ministry of Education
WOS记录号: WOS:000269540700004
Citation statistics:
内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/59005
Appears in Collections:北京大学第一临床医学院_儿科_期刊论文

Files in This Item:

There are no files associated with this item.


作者单位: 1.Peking Univ, Hosp 1, Dept Pediat, Beijing 100034, Peoples R China
2.Peking Univ, Hosp 3, Dept Pediat, Beijing 100083, Peoples R China
3.Fuzhou Gen Hosp, Dept Pediat, Fuzhou 350025, Peoples R China

Recommended Citation:
Fan, Qingfeng,Zhang, Han,Ding, Jie,et al. R168H and V165X mutant podocin might induce different degrees of podocyte injury via different molecular mechanisms[J]. GENES TO CELLS,2009,14(9):1079-1090.
Service
Recommend this item
Sava as my favorate item
Show this item's statistics
Export Endnote File
Google Scholar
Similar articles in Google Scholar
[Fan, Qingfeng]'s Articles
[Zhang, Han]'s Articles
[Ding, Jie]'s Articles
CSDL cross search
Similar articles in CSDL Cross Search
[Fan, Qingfeng]‘s Articles
[Zhang, Han]‘s Articles
[Ding, Jie]‘s Articles
Related Copyright Policies
Null
Social Bookmarking
Add to CiteULike Add to Connotea Add to Del.icio.us Add to Digg Add to Reddit
所有评论 (0)
暂无评论
 
评注功能仅针对注册用户开放,请您登录
您对该条目有什么异议,请填写以下表单,管理员会尽快联系您。
内 容:
Email:  *
单位:
验证码:   刷新
您在IR的使用过程中有什么好的想法或者建议可以反馈给我们。
标 题:
 *
内 容:
Email:  *
验证码:   刷新

Items in IR are protected by copyright, with all rights reserved, unless otherwise indicated.

 

 

Valid XHTML 1.0!
Copyright © 2007-2017  北京大学医学部 - Feedback
Powered by CSpace