|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
CTD_human |
Combined deficiency of dystrophin and beta1 integrin in the cardiac myocyte causes myocardial dysfunction, fibrosis and calcification.
|
18340010 |
2008 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Dystrophin-Deficient Cardiomyopathy.
|
27230049 |
2016 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Heart failure invariably affects patients with various forms of Muscular Dystrophy (MD), but the onset and molecular sequelae of altered structure and function resulting from full-length dystrophin (Dp427) deficiency in MD heart tissue are poorly understood.To better understand the role of dystrophin in cardiomyocyte development and the earliest phase of DMD cardiomyopathy, we studied human cardiomyocytes differentiated from induced pluripotent stem cells (hiPSC-CMs) obtained from the urine of a Deuchenne Muscular Dystrophy (DMD) patient.
|
31049579 |
2020 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
These results indicate that viral infection can influence the severity and penetrance of the cardiomyopathy that occurs in the hearts of dystrophin-deficient individuals.
|
12118246 |
2002 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
The cardiomyopathy of Duchenne's muscular dystrophy and the function of dystrophin.
|
8502196 |
1993 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Although mdx mice are deficient in dystrophin, they only develop mild indicators of cardiomyopathy before 1year-of-age, making therapeutic investigations using this model lengthy.
|
22749475 |
2012 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Dystrophin deficiency also gives rise to considerable complications in the heart, including cardiomyopathy development and arrhythmias.
|
30360568 |
2018 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Duchenne Muscular Dystrophy (DMD) cardiomyopathy is a progressive lethal disease caused by the lack of the dystrophin protein in the heart.
|
28623080 |
2017 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Because genetic analysis cannot determine the correct diagnosis in 35% of DMD/BMD cases, we recommend routine examination of immunostaining patterns of dystrophin in endomyocardial biopsy specimens in patients with cardiomyopathy suspected to be the result of BMD.
|
7900621 |
1995 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
NEW & NOTEWORTHY This study examined the effects of phospholamban ablation on the pathophysiology of cardiomyopathy in dystrophin-deficient mice.
|
30118340 |
2018 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
However, 2.5-7.8% of them may present muscle symptoms and cardiomyopathy, attributed to a reduced production of dystrophin, probably because of skewed patterns of X-chromosome inactivation (XCI).
|
23110537 |
2013 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
The central aim to this work was to find out the possible role of dystrophin and titin along with the TNF-alpha in the pathogenesis of cardiomyopathy.
|
20373002 |
2010 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
In the current preclinical study, we demonstrate the therapeutic potential of sarcospan (SSPN) overexpression to alleviate cardiomyopathy associated with Duchenne muscular dystrophy (DMD) utilizing dystrophin-deficient mdx mice with utrophin haploinsufficiency that more accurately represent the severe disease course of human DMD.
|
31039133 |
2019 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
The loss of dystrophin or its associated proteins results in the development of muscle wasting frequently associated with cardiomyopathy.
|
24645914 |
2014 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Muscular dystrophy, mental retardation and cardiomyopathy not associated with dystrophin deficiency.
|
8784804 |
1996 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
These results show that lack of dystrophin is commonly associated with changes in myocardial features well before the onset of changes of systolic function and overt cardiomyopathy.
|
12875769 |
2003 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Nicorandil, a Nitric Oxide Donor and ATP-Sensitive Potassium Channel Opener, Protects Against Dystrophin-Deficient Cardiomyopathy.
|
26940570 |
2016 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
We and others have shown that mosaic dystrophin expression at the wild-type level, depending on the percentage of dystrophin positive cardiomyocytes, can either delay the onset of or fully prevent cardiomyopathy in dystrophin-null mdx mice.
|
27908661 |
2017 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Dual Therapy Deflazacort/Doxycyclyne Is Better Than Deflazacort Monotherapy to Alleviate Cardiomyopathy in Dystrophin-Deficient mdx Mice.
|
28793824 |
2017 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
CTD_human |
Systemic administration of micro-dystrophin restores cardiac geometry and prevents dobutamine-induced cardiac pump failure.
|
17440445 |
2007 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
We recently identified cleavage and functional impairment of dystrophin by the viral protease 2A during CVB3-infection as a molecular mechanism that may contribute to the pathogenesis of enterovirus-induced cardiomyopathy.
|
11056105 |
2000 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Lack of dystrophin also results to cardiomyopathy, which raises significantly the stroke risk in DMD-patients.
|
29552091 |
2018 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Previously, treatment with β-blockers showed beneficial effects on the development of cardiomyopathy in dystrophin-deficient (mdx) mice, but not in δ-sarcoglycan-deficient (Sgcd-/-) mice.
|
30782477 |
2019 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
HPO |
|
|
|
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Distinct pathophysiological mechanisms of cardiomyopathy in hearts lacking dystrophin or the sarcoglycan complex.
|
21665956 |
2011 |