|
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 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Dystrophin Cardiomyopathies: Clinical Management, Molecular Pathogenesis and Evolution towards Precision Medicine.
|
30235804 |
2018 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
CTD_human |
Intolerance to ß-blockade in a mouse model of δ-sarcoglycan-deficient muscular dystrophy cardiomyopathy.
|
20675662 |
2010 |
|
Cardiomyopathies
|
0.700 |
GeneticVariation
|
group |
BEFREE |
Mutation of dystrophin gene and cardiomyopathy.
|
7981594 |
1994 |
|
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 |
Contrary to previous reports, which indicated the involvement of 5'-end mutations in cardiomyopathies as a result of dystrophin gene alterations, this study shows that despite the apparent concentration of deletions in two regions (5'-end and exons 47 through 49), no general conclusions can be drawn regarding the involvement of specific gene mutations in the development of cardiomyopathy.
|
8989125 |
1996 |
|
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 |
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 |