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Primate Model Carrying LMNA Mutation Develops Dilated Cardiomyopathy

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机构: [1]Kunming Univ Sci & Technol, Inst Primate Translat Med, State Key Lab Primate Biomed Res, Kunming 650500, Yunnan, Peoples R China [2]Yunnan Key Lab Primate Biomed Res, Kunming, Yunnan, Peoples R China [3]Kunming Univ Sci & Technol, Fac Life Sci & Technol, Kunming, Peoples R China [4]Chinese Acad Med Sci & Peking Union Med Coll, Fuwai Hosp, Beijing Key Lab Preclin Res & Evaluat Cardiovasc I, Anim Expt Ctr,Natl Ctr Cardiovasc Dis,Dept Cardiac, 167A Bellishi Rd, Beijing 100037, Peoples R China [5]Chinese Acad Med Sci, Shenzhen Key Lab Cardiovasc Dis, Fuwai Hosp, Shenzhen, Peoples R China [6]NYU, Cardiovasc Res Ctr, Leon H Charney Div Cardiol, Sch Med, New York, NY USA [7]Kunming Univ Sci & Technol, Affiliated Hosp, Peoples Hosp Yunnan Prov 1, Dept Cardiovasc Surg, Kunming, Yunnan, Peoples R China [8]Kunming Univ Sci & Technol, Peoples Hosp Yunnan Prov 1, Yunnan Key Lab Innovat Applicat Tradit Chinese Med, Affiliated Hosp, Kunming, Yunnan, Peoples R China [9]NYU, Dept Neurosci & Physiol, Sch Med, New York, NY USA [10]NYU, Sch Med, Dept Neurol, New York, NY USA
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关键词: CRISPR-mediated adenine base editing dilated cardiomyopathy lamin A/ C primate model

摘要:
To solve the clinical transformation dilemma of lamin A/C (LMNA)-mutated dilated cardiomyopathy (LMD), we developed an LMNA-mutated primate model based on the similarity between the phenotype of primates and humans. We screened out patients with LMD and compared the clinical data of LMD with TTN-mutated and mutation-free dilated cardiomyopathy to obtain the unique phenotype. After establishment of the LMNA c.3572A >G primate model, primates were continuously observed for 48 months, and echocardiographic, electrophysiological, histologic, and transcriptional data were recorded. The LMD primate model was found to highly simulate the phenotype of clinical LMD. In addition, the LMD primate model shared a similar natural history with humans. (c) 2024 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

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大类 | 1 区 医学
小类 | 2 区 心脏和心血管系统
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出版当年[2023]版:
Q1 CARDIAC & CARDIOVASCULAR SYSTEMS
最新[2023]版:
Q1 CARDIAC & CARDIOVASCULAR SYSTEMS

影响因子: 最新[2023版] 最新五年平均 出版当年[2023版] 出版当年五年平均 出版前一年[2022版]

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第一作者机构: [1]Kunming Univ Sci & Technol, Inst Primate Translat Med, State Key Lab Primate Biomed Res, Kunming 650500, Yunnan, Peoples R China [2]Yunnan Key Lab Primate Biomed Res, Kunming, Yunnan, Peoples R China [3]Kunming Univ Sci & Technol, Fac Life Sci & Technol, Kunming, Peoples R China
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通讯机构: [1]Kunming Univ Sci & Technol, Inst Primate Translat Med, State Key Lab Primate Biomed Res, Kunming 650500, Yunnan, Peoples R China [2]Yunnan Key Lab Primate Biomed Res, Kunming, Yunnan, Peoples R China [3]Kunming Univ Sci & Technol, Fac Life Sci & Technol, Kunming, Peoples R China [4]Chinese Acad Med Sci & Peking Union Med Coll, Fuwai Hosp, Beijing Key Lab Preclin Res & Evaluat Cardiovasc I, Anim Expt Ctr,Natl Ctr Cardiovasc Dis,Dept Cardiac, 167A Bellishi Rd, Beijing 100037, Peoples R China [5]Chinese Acad Med Sci, Shenzhen Key Lab Cardiovasc Dis, Fuwai Hosp, Shenzhen, Peoples R China [*1]Beijing Key Laboratory of Preclinical Research and Evaluation for Cardiovascular Implant Materials, Animal Experimental Centre, National Centre for Cardiovascular Disease, Department of Cardiac Surgery, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 167A Bellishi Road, Xi Chen District, Beijing 100037, China. [*2]State Key Laboratory of Primate Biomedical Research,Institute of Primate Translational Medicine, Kunming University of Science and Technology, Kunming, Yunnan 650500, China
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