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Title: Mutations of Cardiac Troponin I cause Restrictive Cardiomyopathy; is cTnI N-terminal deletion an effective rescuing approach ?.
Creator: Getfield, Cecile A., Nan, Changlong, Graduate College, Huang, Xupei
Abstract/Description: Cardiomyopathy is a disorder which affects the heart muscle and causes varied physiological dysfunctions. Restrictive cardiomyopathy RCM is a cardiac muscle disorder in which the left ventricle becomes stiff, due to relaxation impairment. Mutations of the sarcomeric protein cardiac troponin I cTnI gene is found to cause idiopathic RCM. These mutations of cTnI are located in the C-terminus and affect cardiac relaxation. Transgenic mouse models presenting the pathology observed in clinical...
Show moreCardiomyopathy is a disorder which affects the heart muscle and causes varied physiological dysfunctions. Restrictive cardiomyopathy RCM is a cardiac muscle disorder in which the left ventricle becomes stiff, due to relaxation impairment. Mutations of the sarcomeric protein cardiac troponin I cTnI gene is found to cause idiopathic RCM. These mutations of cTnI are located in the C-terminus and affect cardiac relaxation. Transgenic mouse models presenting the pathology observed in clinical patients with RCM have been generated by expressing the mutant cTnI in the heart. RCM-linked mutations increase cardiac myofilament Ca2 sensitivity and promote diastolic dysfunction in the heart. In our laboratory, we have generated double transgenic mice cTnI R193H/ND/KO by crossing the cTnI R193H mice with transgenic cTnI-N terminal truncated mice cTnI-ND. Previous studies using the double transgenic mice showed that ventricular relaxation is enhanced in these mice. This study’s aim is to investigate whether or not the cardiac troponin I N-terminal deletion cTnI-ND corrects the debilitating effects caused by the cTnI R193H high expression in the heart. In doing so, we have first confirmed the genetypes of each experimental group. Echocardiography measurements have been performed on the animals at age 19-21 days. Our data indicate that cTnI R193H/KO showed a significant diastolic dysfunction, whereas the cardiac function in double transgenic mice did not show any difference compared to that in the wild type group, suggesting a functional correction by cTnI-ND in RCM phenotype.

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Date Issued: 2014
PURL: http://purl.flvc.org/fau/fd/FA00005147
Format: Document (PDF)

Title: Dose-dependent arrhythmia and cardiac dysfunction in restrictive cardiomyopathy mice due to troponin mutations.
Creator: Li, Yuejin, Jean-Charles, Pierre-Yves, Nan, Changlong, Chen, Guozhen, Graduate College, Huang, Xupei
Date Issued: 2011-04-08
PURL: http://purl.flvc.org/fcla/dt/3164611
Subject Headings: Arrhythmia --drug therapy, Heart failure, Proteomics
Format: Document (PDF)

Title: Roles of planar cell polarity pathways in the development of neutral tube defects.
Creator: Wu, Gang, Huang, Xupei, Hua, Yimin, Mu, Dezhi
Date Issued: 2011
PURL: http://purl.flvc.org/fau/fd/FAUIR000154
Format: Citation

Title: Sodium valproate-induced congenital cardiac abnormalities in mice are associated with the inhibition of histone deacetylase.
Creator: Wu, Gang, Nan, Changlong, Rollo, Johnathon C, Huang, Xupei, Tian, Jie
Date Issued: 2010
PURL: http://purl.flvc.org/fau/fd/FAUIR000219
Format: Citation

Title: Spatiotemporal expression of histone acetyltransferases, p300 and CBP, in developing embryonic hearts.
Creator: Chen, Guozhen, Zhu, Jing, Lv, Tiewei, Wu, Gang, Sun, Hui-Chao, Huang, Xupei, Tian, Jie
Date Issued: 2009
PURL: http://purl.flvc.org/fau/fd/FAUIR000153
Format: Citation

Title: DNA methylation regulates mouse cardiac myofibril gene expression during heart development.
Creator: Xu, Yang, Liu, Lingjuan, Pan, Bo, Zhu, Jing, Nan, Changlong, Huang, Xupei, Tian, Jie
Date Issued: 2015-12-17
PURL: http://purl.flvc.org/fau/fd/FAUIR000152
Format: Citation

Title: Protective effects of indomethacin and dexamethasone in a goat model with intrauterine balloon aortic valvuloplasty.
Creator: Zhou, Kaiyu, Wu, Gang, Li, Yifei, Zhao, Liang, Zhou, Rong, Zhu, Qi, Huang, Xupei, Mu, Dezhi, Hua, Yimin
Date Issued: 2012
PURL: http://purl.flvc.org/fau/fd/FAUIR000151
Format: Citation

Title: Evaluation of cardiac function in cTnI(R192H) transgenic mice and cTni knockout mice with High-Resolution Ultrasound Imaging and Doppler Echocardiography.
Creator: Liu, Jing, Huang, Xupei, Florida Atlantic University, Charles E. Schmidt College of Medicine, Department of Biomedical Science
Abstract/Description: Troponin I is a contractile protein and plays an important role in cardiac function. We have generated cTnl knockout and cTnI(R192H) transgenic mouse models. All of cTnl knockout homozygous mice die at 17-18 days after birth. Some of cTnI(R192H) transgenic mice die at early life stages, some mice develop heart failure at late stages. High-resolution ultrasound imaging and Doppler echocardiography have been used to evaluate cardiac function on cTnl deficient mice and cTnl(R192H) transgenic...
Show moreTroponin I is a contractile protein and plays an important role in cardiac function. We have generated cTnl knockout and cTnI(R192H) transgenic mouse models. All of cTnl knockout homozygous mice die at 17-18 days after birth. Some of cTnI(R192H) transgenic mice die at early life stages, some mice develop heart failure at late stages. High-resolution ultrasound imaging and Doppler echocardiography have been used to evaluate cardiac function on cTnl deficient mice and cTnl(R192H) transgenic mice. cTnI mice have damaged relaxation with gradually decreased E/A ratio(E/A<1). FS and cardiac output dramatically decrease on 17-day-o1d cTnI mice indicating severe cardiac dysfunction. We find that the damaged heart function is correspondent with the Tnl expression level decline. 6-8 weeks transgenic mice have shown that the dimension of left and right atria increase. In 15-month-old transgenic mice, the E/A ratio shows a pseudonormal pattern indicating a diastolic dysfunction. This study demonstrate that damaged heart function is tightly associated with Tnl levels in the heart.
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Date Issued: 2006
PURL: http://purl.flvc.org/fau/fd/FA00000789
Subject Headings: Transgenic mice, Mice as laboratory animals, Coronary heart disease--Seriodiagnosis, Congestive heart failure--Pathophysiology
Format: Document (PDF)

Title: Energy metabolism and slow skeletal troponin I phosphorylation in cardiac troponin I null mouse heart.
Creator: Jia, Yuanyuan, Florida Atlantic University, Huang, Xupei, Charles E. Schmidt College of Medicine, Department of Biomedical Science
Abstract/Description: Troponin I (TnI) plays an important role in cardiac muscle contraction. Two TnI genes (cardiac and slow skeletal TnI) are predominantly expressed in the heart. In cTnI knockout mice, myocardial TnI deficiency results in a diastolic dysfunction and a sudden death in homozygous mutants. In the present studies, energy metabolism has been analyzed in myocardial cells from cTnI null hearts. Our results have demonstrated that damaged relaxation and increased Ca2+-independent force production in...
Show moreTroponin I (TnI) plays an important role in cardiac muscle contraction. Two TnI genes (cardiac and slow skeletal TnI) are predominantly expressed in the heart. In cTnI knockout mice, myocardial TnI deficiency results in a diastolic dysfunction and a sudden death in homozygous mutants. In the present studies, energy metabolism has been analyzed in myocardial cells from cTnI null hearts. Our results have demonstrated that damaged relaxation and increased Ca2+-independent force production in cTnI null hearts stimulated myofibril MgATPase activities accompanied by the increase of mitochondria quantity and ATPase activities. In addition, an increase of ssTnI phosphorylation level has been observed in cTnI null hearts. The results indicate that TnI deficiency can cause the disturbance of energy metabolism and some protein overphosphorylation.
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Date Issued: 2003
PURL: http://purl.flvc.org/fcla/dt/12998
Subject Headings: Mice as laboratory animals, Mice--Metabolism, Energy metabolism, Mitochondria
Format: Document (PDF)

Title: N-terminal truncated cardiac TnI improves cardiac function in vivo and rescues restrictive cardiomyopathy.
Creator: Jean-Charles, Pierre-Yves, Li, Yuejin, Nan, Changlong, Chen, Guozhen, Feng, H., Huang, Xupei, Jin, J.P., Graduate College
Date Issued: 2011-04-08
PURL: http://purl.flvc.org/fcla/dt/3164515
Subject Headings: Cardiac arrest, Troponin --diagnostic use, Transgenic mice
Format: Document (PDF)

Title: Green tea extract catechin improves internal cardiac muscle relaxation in RCM mice.
Creator: Wang, Xiaoqin, Zhang, Zhengyu, Wu, Gang, Nan, Changlong, Shen, Wen, Hua, Yimin, Huang, Xupei
Date Issued: 2016-12-28
PURL: http://purl.flvc.org/fau/fd/FAUIR000199
Format: Citation

Title: Identification of a truncated form of methionine sulfoxide reductase a expressed in mouse embryonic stem cells.
Creator: Jia, Pingping, Zhang, Chi, Jia, Yuanyuan, Webster, Keith A., Huang, Xupei, Kochegarov, Andrei A., Lemanski, Sharon L., Lemanski, Larry F.
Date Issued: 2011-06-22
PURL: http://purl.flvc.org/fcla/dt/3327268
Subject Headings: Cell nucleus -- metabolism, Cloning, Molecular, Cytosol --metabolism, Embryonic Stem Cells --metabolism, Methionine --metabolism, Methionine Sulfoxide Reductases, Methionine Sulfoxide Reductases --metabolism, Methionine Sulfoxide Reductases --genetics, Mitochondria --metabolism, Molecular Sequence Data, Oxidation --Reduction, Polymerase Chain Reaction, Reverse Transcriptase Polymerase Chain Reaction, Methionine
Format: Document (PDF)

Title: Myofibril-Inducing RNA (MIR) is essential for tropomyosin expression and myofibrillogenesis in axolotl hearts.
Creator: Zhang, Chi, Jia, Pingping, Huang, Xupei, Sferrazza, Gian Franco, Athauda, Gagani, Achary, Mohan P., Wang, Jikui, Lemanski, Sharon L., Lemanski, Larry F.
Date Issued: 2009-09-03
PURL: http://purl.flvc.org/fcla/dt/3327272
Subject Headings: Ambystoma mexicanum --embryology, Ambystoma mexicanum --genetics, Base Sequence, DNA, Complementary --genetics, Endoderm --physiology, Exons --genetics, Gene Expression Regulation, Developmental, Gene Knockdown Techniques, Genes, Recessive, Heart --embryology, Models, Genetic, Molecular Sequence Data, Muscle Proteins --biosynthesis, Muscle Proteins --genetics, Myocardial Contraction --physiology, Protein Isoforms, Reverse Transcriptase Polymerase Chain Reaction, Tropomyosin
Format: Document (PDF)

Title: Inhibition of Histone H3K9 Acetylation by Anacardic Acid Can Correct the Over-Expression of Gata4 in the Hearts of Fetal Mice Exposed to Alcohol during Pregnancy.
Creator: Peng, Chang, Zhu, Jing, Sun, Hui-Chao, Huang, Xupei, Zhao, Wei-An, Zheng, Min, Liu, Lingjuan, Tian, Jie, Dettman, Robert
Date Issued: 2014-08-07
PURL: http://purl.flvc.org/fau/fd/FAUIR000103
Format: Citation

Title: cTnI N-Terminal deletion: an agent for rescuing restrictive cardiomyopathy, a disease caused by mutations of Cardiac Troponin I.
Creator: Getfield, Cecile A., Huang, Xupei, Florida Atlantic University, Charles E. Schmidt College of Medicine, Department of Biomedical Science
Abstract/Description: Restrictive cardiomyopathy (RCM) is represented in part by left ventricular stiffness and diastolic dysfunction. Missense mutations of the cardiac troponin I (cTnI) gene cause idiopathic RCM. These mutations are located in the C-terminus of cTnI and affect cardiac relaxation. Transgenic mouse models presenting the pathology observed in clinical patients with RCM have been generated previously and express the mutant cTnI in their hearts. RCM-linked mutations increase cardiac myofilament Ca2+...
Show moreRestrictive cardiomyopathy (RCM) is represented in part by left ventricular stiffness and diastolic dysfunction. Missense mutations of the cardiac troponin I (cTnI) gene cause idiopathic RCM. These mutations are located in the C-terminus of cTnI and affect cardiac relaxation. Transgenic mouse models presenting the pathology observed in clinical patients with RCM have been generated previously and express the mutant cTnI in their hearts. RCM-linked mutations increase cardiac myofilament Ca2+ sensitivity and promote diastolic dysfunction in the heart. Previous studies using double transgenic mice (cTnI/R193H/ND) showed that ventricular relaxation is enhanced in the cTnI/R193H transgenic mice. In this study, another double transgenic mouse model, (cTnI/R193H/ND/KO), provides an avenue to investigate its rescuing effects on RCMlinked mutations in the cTnI /R193H/KO mouse. Use of molecular biological techniques, transgenic animal developments and murine echocardiography in this study has culminated into a greater understanding of RCM and diastolic dysfunction.
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Date Issued: 2014
PURL: http://purl.flvc.org/fau/fd/FA00004196, http://purl.flvc.org/fau/fd/FA00004196
Subject Headings: Biochemical markers -- Diagnostic use, Cardiovascular system -- Pathophysiology, Coronary heart disease -- Molecular diagnosis, Mice as laboratory animals, Molecular biology
Format: Document (PDF)

Title: Muscle Contractility and Cell Motility.
Creator: Jin, J.P., Bloch, Robert J., Huang, Xupei, Larsson, Lars
Date Issued: 2012
PURL: http://purl.flvc.org/fau/fd/FAUIR000102
Format: Citation

Title: Planar Cell Polarity Signaling Pathway in Congenital Heart Diseases.
Creator: Wu, Gang, Ge, Jiao, Huang, Xupei, Hua, Yimin, Mu, Dezhi
Date Issued: 2011
PURL: http://purl.flvc.org/fau/fd/FAUIR000155
Format: Citation

FAU College Collections (11)	+ -
FAU Theses and Dissertations (3)	+ -
FAU Graduate Student Research (3)	+ -

Mice as laboratory animals (3)	+ -
Molecular Sequence Data (2)	+ -
Reverse Transcriptase Polymerase Chain Reaction (2)	+ -
Transgenic mice (2)	+ -
Ambystoma mexicanum --embryology (1)	+ -

Ambystoma mexicanum --genetics (1)	+ -
Arrhythmia --drug therapy (1)	+ -
Base Sequence (1)	+ -
Biochemical markers -- Diagnostic use (1)	+ -
Cardiac arrest (1)	+ -
Cardiovascular system -- Pathophysiology (1)	+ -
Cell nucleus -- metabolism (1)	+ -
Cloning, Molecular (1)	+ -
Congestive heart failure--Pathophysiology (1)	+ -
Coronary heart disease -- Molecular diagnosis (1)	+ -
Coronary heart disease--Seriodiagnosis (1)	+ -
Cytosol --metabolism (1)	+ -
DNA, Complementary --genetics (1)	+ -
Embryonic Stem Cells --metabolism (1)	+ -
Endoderm --physiology (1)	+ -
Energy metabolism (1)	+ -
Exons --genetics (1)	+ -
Gene Expression Regulation, Developmental (1)	+ -
Gene Knockdown Techniques (1)	+ -
Genes, Recessive (1)	+ -
Heart --embryology (1)	+ -
Heart failure (1)	+ -
Methionine (1)	+ -
Methionine --metabolism (1)	+ -
Methionine Sulfoxide Reductases (1)	+ -

article (9)	+ -
Electronic Thesis or Dissertation (3)	+ -
abstract (2)	+ -
poster (1)	+ -

Huang, Xupei (17)	+ -
Nan, Changlong (6)	+ -
Wu, Gang (6)	+ -
Hua, Yimin (4)	+ -
Tian, Jie (4)	+ -

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