|
Muscle degeneration
|
0.100 |
Biomarker
|
disease |
BEFREE |
Dystrophin deficiency leads to progressive muscle degeneration in Duchenne muscular dystrophy (DMD) patients.
|
30944252 |
2019 |
|
Muscle degeneration
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Duchenne muscular dystrophy (DMD) is a genetic condition caused by mutations in the DMD gene leading to muscle degeneration, fatty replacement of muscle cells and fibrosis.
|
30394578 |
2019 |
|
Muscle degeneration
|
0.100 |
Biomarker
|
disease |
BEFREE |
However, its role in DMD has not been studied so far.In our work, we have generated mice globally lacking both dystrophin and miR-146a (miR-146a<sup>-/-</sup>mdx) and examined them together with wild-type, single miR-146a knockout and dystrophic (mdx-lacking dystrophin) mice in a variety of aspects associated with DMD pathophysiology (muscle degeneration, inflammatory reaction, muscle satellite cells, muscle regeneration, and fibrosis).We have shown that miR-146a level is increased in dystrophic muscles in comparison to wild-type mice.
|
31412923 |
2019 |
|
Muscle degeneration
|
0.100 |
Biomarker
|
disease |
BEFREE |
Duchenne muscular dystrophy (DMD), caused by dystrophin deficiency, results in chronic inflammation and irreversible skeletal muscle degeneration.
|
30074247 |
2019 |
|
Muscle degeneration
|
0.100 |
Biomarker
|
disease |
BEFREE |
Duchenne muscular dystrophy (DMD) is a lethal X-linked recessive pathology in which lack of functional dystrophin leads to progressive muscle degeneration culminating in loss of locomotion and premature death.
|
30912260 |
2019 |
|
Muscle degeneration
|
0.100 |
Biomarker
|
disease |
BEFREE |
Boys with Duchenne muscular dystrophy (DMD) have DMD gene mutations, with associated loss of the dystrophin protein and progressive muscle degeneration and weakness.
|
29843823 |
2018 |
|
Muscle degeneration
|
0.100 |
Biomarker
|
disease |
BEFREE |
We found that: (i) dystrophin-dependent muscle degeneration was accompanied by a drastic increase in mitochondrial fragmentation that can be rescued by genetic manipulations of mitochondrial dynamics (ii) the loss of function of the fission gene drp-1 or the overexpression of the fusion genes eat-3 and fzo-1 provoked a reduction of muscle degeneration and an improved mobility of dystrophin mutant worms (iii) the functions of DRP-1 in apoptosis and of others apoptosis executors are important for dystrophin-dependent muscle cell death (iv) DRP-1-mediated apoptosis is also likely to induce age-dependent loss of muscle cell.
|
29743663 |
2018 |
|
Muscle degeneration
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Duchenne muscular dystrophy (DMD) is a genetic disease associated with mutations of Dystrophin gene that regulate myofiber integrity and muscle degeneration, characterized by oxidative stress increase.
|
29636844 |
2018 |
|
Muscle degeneration
|
0.100 |
Biomarker
|
disease |
BEFREE |
Genetic disruption of the dystrophin complex produces muscular dystrophy characterized by a fragile muscle plasma membrane leading to excessive muscle degeneration.
|
29065150 |
2017 |
|
Muscle degeneration
|
0.100 |
Biomarker
|
disease |
BEFREE |
Absence of dystrophin protein from the sarcolemma causes severe muscle degeneration, fibrosis, and inflammation, ultimately leading to cardiorespiratory failure and premature death.
|
28533404 |
2017 |
|
Muscle degeneration
|
0.100 |
Biomarker
|
disease |
BEFREE |
Nevertheless, the mechanisms by which the absence of dystrophin leads to muscle degeneration remain to be fully elucidated.
|
28845212 |
2017 |
|
Muscle degeneration
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
In Duchenne muscular dystrophy (DMD), lack of dystrophin leads to progressive muscle degeneration, with DMD patients suffering from cardiorespiratory failure.
|
28623422 |
2017 |
|
Muscle degeneration
|
0.100 |
GeneticVariation
|
disease |
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 |
|
Muscle degeneration
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
In Duchenne muscular dystrophy (DMD), the loss of the dystrophin component of the dystrophin-glycoprotein complex (DGC) compromises plasma membrane integrity in skeletal muscle, resulting in extensive muscle degeneration.
|
28806929 |
2017 |
|
Muscle degeneration
|
0.100 |
Biomarker
|
disease |
BEFREE |
Duchenne muscular dystrophy (DMD) is a genetic disorder in which the absence of dystrophin leads to progressive muscle degeneration and weakness.
|
27979987 |
2017 |
|
Muscle degeneration
|
0.100 |
Biomarker
|
disease |
BEFREE |
Because of those activities on different steps of muscle regeneration, we hypothesized a beneficial effect of UnAG in mdx dystrophic mice, in which the absence of dystrophin leads to chronic muscle degeneration, defective muscle regeneration, fibrosis, and, at later stages of the pathology, SC pool exhaustion.
|
28436144 |
2017 |
|
Muscle degeneration
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
In Duchenne muscular dystrophy (DMD), NF-κB is activated in skeletal muscle from infancy regardless of the underlying dystrophin mutation and drives inflammation and muscle degeneration while inhibiting muscle regeneration.
|
28074489 |
2017 |
|
Muscle degeneration
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Absence or reduced expression of dystrophin or many of the DPC components cause the muscular dystrophies, a group of inherited diseases in which repeated bouts of muscle damage lead to atrophy and fibrosis, and eventually muscle degeneration.
|
26676145 |
2016 |
|
Muscle degeneration
|
0.100 |
Biomarker
|
disease |
BEFREE |
By studying a Caenorhabditis elegans model for DMD, we show here that dystrophin-dependent muscle degeneration is likely to be cell autonomous and affects the muscle cells the most involved in locomotion.
|
26358775 |
2015 |
|
Muscle degeneration
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Duchenne muscular dystrophy (DMD), caused by a lack of the functional structural protein dystrophin, leads to severe muscle degeneration where the patients are typically wheelchair-bound and die in their mid-twenties from cardiac or respiratory failure or both.
|
26316011 |
2015 |
|
Muscle degeneration
|
0.100 |
Biomarker
|
disease |
BEFREE |
Moreover, CRISPR/Cas9 induced mutations in both male and female monkeys, with the markedly depleted dystrophin and muscle degeneration seen in early DMD.
|
25859012 |
2015 |
|
Muscle degeneration
|
0.100 |
Biomarker
|
disease |
BEFREE |
By pharmacologically modulating the expression of the dystrophin-related protein utrophin, we have previously demonstrated in dystrophin-deficient mdx studies, daily SMT C1100 treatment significantly reduced muscle degeneration leading to improved muscle function.
|
25935002 |
2015 |
|
Muscle degeneration
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Duchenne muscular dystrophy (DMD) is an X-linked recessive disease caused by mutations in the dystrophin gene and is characterized by muscle degeneration and death.
|
24135430 |
2014 |
|
Muscle degeneration
|
0.100 |
Biomarker
|
disease |
BEFREE |
We find that the muscle degeneration observed in a C. elegans model of dystrophin-based muscular dystrophy can be suppressed by clp-1 inactivation and that nemadipine-A inhibition of the EGL-19 calcium channel reveals that Ca(2+) dysfunction underlies the C. elegans MyoD model of myopathy.
|
22479198 |
2012 |
|
Muscle degeneration
|
0.100 |
Biomarker
|
disease |
BEFREE |
This lack of dystrophin leads to the progressive muscle degeneration that is often responsible for the death of the DMD patients during the third decade of their life.
|
21468001 |
2011 |