Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Dystrophin-deficient cardiomyopathy is becoming the dominant cause of death in patients with Duchenne muscular dystrophy (DMD), but its developmental process remains elusive.
|
31611157 |
2020 |
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 |
AlteredExpression
|
group |
BEFREE |
Improving skeletal muscle function without restoring dystrophin expression in cardiac tissue may exacerbate cardiomyopathy due to increased voluntary activity.
|
30972156 |
2019 |
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 |
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 |
Dystrophin deficiency leads to ambulation loss and cardiomyopathy.
|
30518686 |
2018 |
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Although this minigene fully normalized skeletal muscle force, it only partially corrected electrocardiogram and heart hemodynamics in dystrophin-null mdx mice that had moderate cardiomyopathy.
|
29433343 |
2018 |
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 |
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 |
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 |
AlteredExpression
|
group |
BEFREE |
In conclusion, this study demonstrated that allogeneic WT-MPC-Exo transplantation transiently restored dystrophin gene expression and improved cardiac function in MDX mice, suggesting that allogenic exosomal delivery may serve as an alternative treatment for cardiomyopathy of DMD.
|
30155598 |
2018 |
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 |
Dystrophin Cardiomyopathies: Clinical Management, Molecular Pathogenesis and Evolution towards Precision Medicine.
|
30235804 |
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 |
GeneticVariation
|
group |
BEFREE |
Duchenne muscular dystrophy (DMD) is caused by abnormalities in the dystrophin gene and is clinically characterised by childhood muscle degeneration and cardiomyopathy.
|
29127875 |
2017 |
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 |
BEFREE |
Dystrophin-Deficient Cardiomyopathy.
|
27230049 |
2016 |
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 |
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 |
Proteomic profiling of the dystrophin-deficient mdx phenocopy of dystrophinopathy-associated cardiomyopathy.
|
24772416 |
2014 |
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 |
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 |
Dystrophin-deficient Duchenne cardiomyopathy is associated with Duchenne muscular dystrophy (DMD), the most common lethal muscular dystrophy.
|
22318092 |
2012 |
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 |