Abstract 773: Neonatal and Adult Cardiac Transcriptome Analysis Reveals Distinct Mechanisms in Noncompaction Dilated Cardiomyopathy Development Induced by p.K69R-MLP (Muscle Lim Protein) Mutation i n vivo

2019 ◽  
Vol 125 (Suppl_1) ◽  
Author(s):  
Enkhsaikhan Purevjav ◽  
Fuyi Xu ◽  
Undral Munkhsaikhan ◽  
Neely Alberson ◽  
Ramona Sabau ◽  
...  
Keyword(s):  
Dilated Cardiomyopathy ◽  
Transcriptome Analysis ◽  
Lim Protein ◽  
Protein Mutation ◽  
Muscle Lim Protein

Mutations in the muscle LIM protein and α-actinin-2 genes in dilated cardiomyopathy and endocardial fibroelastosis

2003 ◽  
Vol 80 (1-2) ◽  
pp. 207-215 ◽  
Author(s):  
Bhagyalaxmi Mohapatra ◽  
Shinawe Jimenez ◽  
Jiuann Huey Lin ◽  
Karla R Bowles ◽  
Karen J Coveler ◽  
...  
Keyword(s):  
Dilated Cardiomyopathy ◽  
Endocardial Fibroelastosis ◽  
Lim Protein ◽  
Muscle Lim Protein

Muscle LIM protein deficiency leads to alterations in passive ventricular mechanics

2002 ◽  
Vol 282 (2) ◽  
pp. H680-H687 ◽  
Author(s):  
Jeffrey H. Omens ◽  
Taras P. Usyk ◽  
Zuangjie Li ◽  
Andrew D. McCulloch
Keyword(s):  
Heart Failure ◽  
Dilated Cardiomyopathy ◽  
Three Dimensional ◽  
Clinical Signs ◽  
Targeted Disruption ◽  
Lim Protein ◽  
Ventricular Mechanics ◽  
Muscle Lim Protein ◽  
Important Pathway ◽  
Knockout Model

Accumulating evidence indicates that cytoskeletal defects may be an important pathway for dilated cardiomyopathy and eventual heart failure. Targeted disruption of muscle LIM protein (MLP) has previously been shown to result in dilated cardiomyopathy with many of the clinical signs of heart failure, although the effects of MLP disruption on passive ventricular mechanics and myocyte architecture are not known. We used the MLP knockout model to examine changes in passive ventricular mechanics and laminar myofiber sheet architecture. Pressure-volume and pressure-strain relations were altered in MLP knockout mice, in general suggesting a less compliant tissue in the dilated hearts. Transmural laminar myocyte structure was also altered in this mouse model, especially near the epicardium. A mathematical model of the heart showed a likely increase in passive tissue stiffness in the MLP-deficient (−/−) heart. These results suggest that the disruption of the cytoskeletal protein MLP results in less compliant passive tissue and concomitant structural alterations in the three-dimensional myocyte architecture that may in part explain the ventricular dysfunction in the dilated heart.

scholarly journals Muscle LIM protein/CSRP3: a mechanosensor with a role in autophagy

2015 ◽  
Vol 1 (1) ◽  
Author(s):  
M M Rashid ◽  
A Runci ◽  
L Polletta ◽  
I Carnevale ◽  
E Morgante ◽  
...  
Keyword(s):  
Lim Protein ◽  
Muscle Lim Protein

Dilated cardiomyopathy may be an early sign of the C826A Fukutin-related protein mutation

2005 ◽  
Vol 15 (5) ◽  
pp. 372-376 ◽  
Author(s):  
T. Müller ◽  
M. Krasnianski ◽  
R. Witthaut ◽  
M. Deschauer ◽  
S. Zierz
Keyword(s):  
Dilated Cardiomyopathy ◽  
Early Sign ◽  
Related Protein ◽  
Protein Mutation
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