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学科主题: 基础医学
题名:
Deficiency of cartilage oligomeric matrix protein causes dilated cardiomyopathy
作者: Huang, Yaqian1; Xia, Jiahong2; Zheng, Jingang3; Geng, Bin1; Liu, Peng3; Yu, Fang1; Liu, Bo1; Zhang, Hongquan4; Xu, Mingjiang1; Ye, Ping2; Zhu, Yi1; Xu, Qingbo5; Wang, Xian1; Kong, Wei1,6
关键词: Dilated cardiomyopathy ; Matricellular protein ; Integrin ; Ubiquitination
刊名: BASIC RESEARCH IN CARDIOLOGY
发表日期: 2013-09-01
DOI: 10.1007/s00395-013-0374-9
卷: 108, 期:5
收录类别: SCI
文章类型: Article
WOS标题词: Science & Technology
类目[WOS]: Cardiac & Cardiovascular Systems
研究领域[WOS]: Cardiovascular System & Cardiology
关键词[WOS]: FOCAL ADHESION KINASE ; CARDIAC-HYPERTROPHY ; HEART-FAILURE ; PRESSURE-OVERLOAD ; TARGETED ABLATION ; INTEGRIN ; MICE ; GENE ; MUTATIONS ; DISEASE
英文摘要:

Alterations in cardiac extracellular matrix are involved in dilated cardiomyopathy (DCM) and its progression to heart failure. The matricellular protein cartilage oligomeric matrix protein (COMP) has been indicated localized in the heart. However, the role of COMP in cardiac homeostasis and disease remains elusive. COMP-/- mice, both male and female, developed DCM spontaneously at young age (3-5 months), with impaired cardiac function. Assessment of postnatal COMP-/- heart at 1 month, although functionally normal, revealed severe cardiac ultrastructure defect, in parallel with cardiomyocyte apoptosis, myofilament loss, connexin-43 deficiency and matrix metalloproteinase activation. Decreased COMP expression was observed in the heart sample of DCM patients compared with donor heart. Mechanistically, COMP-/- heart exhibited reduced integrin beta 1 expression and signaling. Ectopic expression of COMP or integrin beta 1 rescued COMP-deficiency- induced cardiomyocyte apoptosis, myofilament dissolution, and connexin-43 aberrance. Additionally, COMP directly bonded to the extracellular beta-tail domain of integrin beta 1, prevented integrin beta 1 ubiquitination/ degradation, and maintained the cardiac homeostasis. COMP-integrin beta 1 axis is a potential target of DCM.

语种: 英语
所属项目编号: 81225002 ; 81220108004 ; 2012CB518002 ; 81070243 ; B07001
项目资助者: National Science Fund for Distinguished Young Scholars ; Projects of International Cooperation and Exchanges NSFC ; National Program on Key Basic Research Project (973 Program) ; National Natural Science Foundation of the P.R. China ; "111" Project of Chinese Ministry of Education
WOS记录号: WOS:000324212500006
Citation statistics:
内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/53529
Appears in Collections:基础医学院_期刊论文

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作者单位: 1.Minist Educ, Key Lab Mol Cardiovasc Sci, Beijing 100191, Peoples R China
2.Peking Univ, Basic Med Coll, Dept Physiol & Pathophysiol, Beijing 100191, Peoples R China
3.Huazhong Univ Sci Technol, Union Hosp, Tongji Med Coll, Dept Cardiovasc Surg, Wuhan, Peoples R China
4.China Japan Friendship Hosp, Dept Cardiol, Beijing 100029, Peoples R China
5.Peking Univ, Hlth Sci Ctr, Dept Anat Histol & Embryol, Beijing 100191, Peoples R China
6.Kings Coll London, James Black Ctr, Cardiovasc Div, London SE5 9NU, England

Recommended Citation:
Huang, Yaqian,Xia, Jiahong,Zheng, Jingang,et al. Deficiency of cartilage oligomeric matrix protein causes dilated cardiomyopathy[J]. BASIC RESEARCH IN CARDIOLOGY,2013,108(5).
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