|
Cardiomyopathies
|
0.700 |
GeneticVariation
|
group |
BEFREE |
Young males presenting with apparent isolated cardiomyopathy or acute myocarditis may harbor dystrophin mutations without overt skeletal muscle pathology.
|
20206892 |
2010 |
|
Cardiomyopathies
|
0.700 |
GeneticVariation
|
group |
BEFREE |
X-chromosome inactivation was shown to be the basis of cardiomyopathy in women with a single mutated dystrophin allele.
|
7787263 |
1995 |
|
Cardiomyopathies
|
0.700 |
GeneticVariation
|
group |
BEFREE |
We report the first case of a DNA segment deletion encompassing promoters M and P of the human dystrophin gene, which caused a very severe muscle phenotype without cardiomyopathy, in a 13-year-old boy.
|
12031623 |
2002 |
|
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 |
GeneticVariation
|
group |
BEFREE |
We identified a novel dystrophin mutation (p.1667 del Ala), resulting in BMD-associated cardiomyopathy that demonstrated the pathological features of significant fibrofatty replacement in the sub-epicardial layer of the ventricle; further, the high-throughput sequencing is helpful for making an early diagnosis of BMD.
|
30103083 |
2018 |
|
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 |
GeneticVariation
|
group |
BEFREE |
We analyzed 78 BMD and X-linked dilated cardiomyopathy patients with common deletion mutations predicted to alter the dystrophin protein and correlated their mutations to cardiomyopathy age of onset.
|
20031633 |
2009 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Thus current research efforts into the elucidation of the molecular mechanisms underlying these genetic diseases are not only directed towards studying skeletal muscle necrosis but also investigate abnormalities of heart and brain dystrophin-glycoprotein complexes in cardiomyopathy and brain deficiencies associated with muscular dystrophy.
|
9850730 |
1998 |
|
Cardiomyopathies
|
0.700 |
AlteredExpression
|
group |
BEFREE |
This work demonstrates that high levels of cardiac dystrophin restored by Pip peptide-AOs prevents further deterioration of cardiomyopathy and pathology following exercise in dystrophic DMD mice.
|
25758104 |
2015 |
|
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 |
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 |
These findings indicate that AAV-mediated cardiac transduction with microdystrophin might be a promising therapeutic strategy for the treatment of dystrophin-deficient cardiomyopathy.
|
21451578 |
2011 |
|
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 |
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 |
AlteredExpression
|
group |
BEFREE |
The absence of dystrophin and the down-regulation of the dystrophin-associated proteins in the heart accounted for the severe cardiomyopathy in this family.
|
7635962 |
1995 |
|
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 |
CTD_human |
Systemic administration of micro-dystrophin restores cardiac geometry and prevents dobutamine-induced cardiac pump failure.
|
17440445 |
2007 |
|
Cardiomyopathies
|
0.700 |
Therapeutic
|
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 |
Proteomic profiling of the dystrophin-deficient mdx phenocopy of dystrophinopathy-associated cardiomyopathy.
|
24772416 |
2014 |
|
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 |
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 |
NEW & NOTEWORTHY This study examined the effects of phospholamban ablation on the pathophysiology of cardiomyopathy in dystrophin-deficient mice.
|
30118340 |
2018 |
|
Cardiomyopathies
|
0.700 |
GeneticVariation
|
group |
BEFREE |
Mutations of the dystrophin gene leading to a complete loss of the protein cause Duchenne muscular dystrophy (DMD), frequently associated with severe cardiomyopathy.
|
22248393 |
2012 |
|
Cardiomyopathies
|
0.700 |
GeneticVariation
|
group |
BEFREE |
Mutation of dystrophin gene and cardiomyopathy.
|
7981594 |
1994 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Muscular dystrophy, mental retardation and cardiomyopathy not associated with dystrophin deficiency.
|
8784804 |
1996 |