Cited by 181Cardiomyopathy and Myosin StudiesCardiac Fibrosis and RemodelingCardiac electrophysiology and arrhythmias

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A transgenic rabbit model for human hypertrophic cardiomyopathy

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Marian, Ali J., Wu, Yun, Lim, Do-Sun, McCluggage, Meghan, Youker, Keith, Yu, Qun-tao, Brugada, Ramon, DeMayo, Francesco, Quinones, Miguel, Roberts, Robert·1999·Journal of Clinical Investigation

DOI 10.1172/jci7956PMC PMC409884

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Abstract

Certain mutations in genes for sarcomeric proteins cause hypertrophic cardiomyopathy (HCM). We have developed a transgenic rabbit model for HCM caused by a common point mutation in the beta-myosin heavy chain (MyHC) gene, R400Q. Wild-type and mutant human beta-MyHC cDNAs were cloned 3' to a 7-kb murine beta-MyHC promoter. We injected purified transgenes into fertilized zygotes to generate two lines each of the wild-type and mutant transgenic rabbits. Expression of transgene mRNA and protein were confirmed by Northern blotting and 2-dimensional gel electrophoresis followed by immunoblotting, respectively. Animals carrying the mutant transgene showed substantial myocyte disarray and a 3-fold increase in interstitial collagen expression in their myocardia. Mean septal thicknesses were comparable between rabbits carrying the wild type transgene and their nontransgenic littermates (NLMs) but were significantly increased in the mutant transgenic animals. Posterior wall thickness and left ventricular mass were also increased, but dimensions and systolic function were normal. Premature death was more common in mutant than in wild-type transgenic rabbits or in NLMs. Thus, cardiac expression of beta-MyHC-Q(403) in transgenic rabbits induced hypertrophy, myocyte and myofibrillar disarray, interstitial fibrosis, and premature death, phenotypes observed in humans patients with HCM due to beta-MyHC-Q(403).

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Authors: Marian, A. J., Wu, Y., Lim, D.-S., McCluggage, M., Youker, K., Yu, Q.-T., Brugada, R., DeMayo, F., Quinones, M., & Roberts, R.
Affiliations: Baylor College of Medicine (US)
ORCID: Marian, Ali J.
Lim, Do-Sun
Youker, Keith
DeMayo, Francesco
Venue: Journal of Clinical Investigation
Publisher: American Society for Clinical Investigation
Published: 1999-12-15
Volume: 104
Issue: 12
Pages: 1683-1692
ISSN: 0021-9738 (ISSN-L)
DOI: 10.1172/jci7956
PMC: PMC409884
OpenAlex: W2123240976
Cited by: 181
References: 62
Citations by year: 2025: 3 · 2024: 3 · 2023: 3 · 2022: 2 · 2021: 6 · 2020: 5 · 2019: 7 · 2018: 2 · 2017: 4 · 2016: 8 · 2015: 2 · 2014: 6 · 2013: 5 · 2012: 6
FWCI: 9.24
Type: journal article
Language: en
Open access: bronze
doi.org
Locations: Journal of Clinical Investigation (publishedVersion)
PubMed Central (publishedVersion)
PubMed (publishedVersion)
Funders: American Heart Association
National Institutes of Health (5p50hl042267-04)
National Heart, Lung, and Blood Institute (P50-HL-42267-01)
Topics: Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiomyopathy and Myosin Studies
Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Fibrosis and Remodeling
Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
Keywords: hypertrophic cardiomyopathy, beta-myosin heavy chain, transgenic rabbit model, sarcomeric protein mutations, myocyte disarray, interstitial fibrosis, left ventricular hypertrophy, premature mortality
MeSH terms: Animals, Cardiomyopathy, Hypertrophic, Collagen, Disease Models, Animal, Humans, Rabbits, RNA, Messenger, Myosin Heavy Chains, Animals, Genetically Modified

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A transgenic rabbit model for human hypertrophic cardiomyopathy

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