Congestive heart failure
|
0.300 |
Biomarker
|
disease |
BEFREE |
In summary, MLP-deficient hESC-CMs recapitulate the pathogenesis of HCM and its progression toward HF, providing an important human model for investigation of CSRP3/MLP-associated disease pathogenesis.
|
31406109 |
2019 |
Congestive heart failure
|
0.300 |
Biomarker
|
disease |
BEFREE |
Mutations in CSRP3 gene cause cardiomyopathies and deregulation of CSRP3 has been found in patients with heart failure and several skeletal muscle diseases.
|
30826063 |
2019 |
Congestive heart failure
|
0.300 |
Biomarker
|
disease |
BEFREE |
Conversely, gene-deleted mice lacking both Mg29 and MLP protein showed a further reduction in t-tubule organization and accelerated heart failure.
|
28706255 |
2017 |
Congestive heart failure
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
Our results suggest that the main role of MLP in heart lies in the direct inhibition of PKCα and that chronic uninhibited PKCα activity at the intercalated disc in the absence of functional MLP leads to heart failure.
|
27353086 |
2016 |
Congestive heart failure
|
0.300 |
Biomarker
|
disease |
BEFREE |
Up-regulation was evident for muscle LIM protein (Mlp), desmin, and heart failure (natriuretic peptide A [Nppa], Nppb, and myosin heavy chain 6) and fibrosis (transforming growth factor beta 1, alpha-smooth muscle actin, osteopontin, and periostin) markers.
|
25541130 |
2014 |
Congestive heart failure
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
Missense mutations in human MLP or its ablation in transgenic mice promotes cardiomyopathy and heart failure.
|
24934443 |
2014 |
Congestive heart failure
|
0.300 |
Biomarker
|
disease |
BEFREE |
Interestingly, MLP was also found to be down-regulated in humans with heart failure (Zolk et al.
|
21484537 |
2011 |
Congestive heart failure
|
0.300 |
Biomarker
|
disease |
BEFREE |
We have further investigated the muscle LIM protein knockout model of HF (MLP-/-), which closely parallels human dilated cardiomyopathy disease progression and aberrant beta-adrenergic signaling, and their betaARKct rescue.
|
17971502 |
2008 |
Congestive heart failure
|
0.300 |
Biomarker
|
disease |
BEFREE |
An MLP-deficient mouse has been described; these mice develop dilated cardiomyopathy and heart failure.
|
14567970 |
2004 |
Congestive heart failure
|
0.300 |
Biomarker
|
disease |
BEFREE |
MLP immunoreactivity was decreased approximately 50% (P<0.05) in the left ventricular myocardium of patients with chronic heart failure due to dilated or ischemic cardiomyopathy compared with non-failing donor hearts.
|
10851202 |
2000 |
Congestive heart failure
|
0.300 |
Biomarker
|
disease |
BEFREE |
To investigate whether alterations in betaAR function contribute to the development of myocardial failure, transgenic mice with cardiac-restricted overexpression of either a peptide inhibitor of betaARK1 or the beta2AR were mated into a genetic model of murine heart failure (MLP-/-).
|
9618528 |
1998 |
Congestive heart failure
|
0.300 |
Biomarker
|
disease |
BEFREE |
In vivo analysis revealed that MLP-deficient mice reproduce the morphological and clinical picture of dilated cardiomyopathy and heart failure in humans, providing the first model for this condition in a genetically manipulatable organism.
|
9039266 |
1997 |
Congestive heart failure
|
0.300 |
Biomarker
|
disease |
MGD |
|
|
|