Cardiomyopathies
|
0.700 |
GeneticVariation
|
group |
BEFREE |
Loss-of-function mutations in the genes encoding dystrophin and the associated membrane proteins, the sarcoglycans, produce muscular dystrophy and cardiomyopathy.
|
21138941 |
2011 |
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Left ventricular thrombosis and systemic emboli have been demonstrated to complicate cardiomyopathy in Duchenne and Becker muscular dystrophy (DMD, BMD).
|
10235436 |
1999 |
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 |
Therapeutic
|
group |
CTD_human |
Intolerance to ß-blockade in a mouse model of δ-sarcoglycan-deficient muscular dystrophy cardiomyopathy.
|
20675662 |
2010 |
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 |
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 |
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 |
In conclusion, low glutathione resource hastens the onset of cardiomyopathy linked to a defect in dystrophin in mdx mice.
|
20696779 |
2010 |
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 |
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 |
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 |
GeneticVariation
|
group |
BEFREE |
Females with dystrophin mutations are at risk for cardiomyopathy, but are usually asymptomatic during childhood.
|
19449433 |
2009 |
Cardiomyopathies
|
0.700 |
GeneticVariation
|
group |
BEFREE |
Duchenne muscular dystrophy (DMD), caused by mutations in the dystrophin gene, is associated with severe cardiac complications including cardiomyopathy and cardiac arrhythmias.
|
21677768 |
2011 |
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 |
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 |
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 |
Distinct pathophysiological mechanisms of cardiomyopathy in hearts lacking dystrophin or the sarcoglycan complex.
|
21665956 |
2011 |
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 |
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 |
Therapeutic
|
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 |
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 |
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 |
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 |
All dystrophin-associated proteins are decreased in abundance in the cardiomyopathic hamster heart, perhaps explaining why the cardiomyopathy is more severe than the myopathy.
|
8505286 |
1993 |
Cardiomyopathies
|
0.700 |
GeneticVariation
|
group |
BEFREE |
A 5' dystrophin duplication mutation causes membrane deficiency of alpha-dystroglycan in a family with X-linked cardiomyopathy.
|
9441825 |
1997 |