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Recent advancements in the molecular genetics of left ventricular noncompaction cardiomyopathy.

Authors
  • Dong, Xueqi1
  • Fan, Peng2
  • Tian, Tao3
  • Yang, Yankun4
  • Xiao, Yan5
  • Yang, Kunqi6
  • Liu, Yaxin7
  • Zhou, Xianliang8
  • 1 Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 167, Beilishi Road, Beijing 100037, China. Electronic address: [email protected]
  • 2 Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 167, Beilishi Road, Beijing 100037, China. Electronic address: [email protected]
  • 3 Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 167, Beilishi Road, Beijing 100037, China. Electronic address: [email protected]
  • 4 Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 167, Beilishi Road, Beijing 100037, China. Electronic address: [email protected]
  • 5 Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 167, Beilishi Road, Beijing 100037, China. Electronic address: [email protected]
  • 6 Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 167, Beilishi Road, Beijing 100037, China. Electronic address: [email protected]
  • 7 Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 167, Beilishi Road, Beijing 100037, China. Electronic address: [email protected]
  • 8 Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 167, Beilishi Road, Beijing 100037, China. Electronic address: [email protected]
Type
Published Article
Journal
Clinica chimica acta; international journal of clinical chemistry
Publication Date
Dec 15, 2016
Volume
465
Pages
40–44
Identifiers
DOI: 10.1016/j.cca.2016.12.013
PMID: 27989498
Source
Medline
Keywords
License
Unknown

Abstract

Left ventricular noncompaction cardiomyopathy (LVNC) is a myocardial disorder characterized by prominent and excessive trabeculations with deep recesses in the ventricular wall. Clinical manifestations of LVNC are highly variable, ranging from no symptoms to arrhythmias, heart failure, thromboembolism, or even sudden cardiac death. It is a heterogenetic disease which can be presented as an autosomal, X-linked or mitochondrial disorder. A series of candidate mutations have been identified in LVNC patients or murine models. It is generally believed that these gene mutations may share a final common pathway in the pathogenesis of LVNC, but the underlying molecular mechanisms are unknown. In this review, we discuss the gene mutations identified in LVNC patients and summarize recent advancements in the molecular genetic analysis of LVNC.

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