Dystrophin Deficiency Causes Progressive Depletion of Cardiovascular Progenitor Cells in the Heart.
Aging
/ genetics
Animals
Cardiomyopathy, Dilated
/ genetics
Cardiovascular System
/ metabolism
DNA Damage
/ genetics
Disease Models, Animal
Dystrophin
/ deficiency
Gene Expression Regulation
/ genetics
Humans
Mice
Mice, Inbred mdx
/ genetics
Muscular Dystrophy, Duchenne
/ genetics
Myocardium
/ metabolism
Myocytes, Cardiac
/ metabolism
Proto-Oncogene Proteins c-kit
/ genetics
Stem Cells
/ metabolism
c-kit
cardiovascular progenitors
dilated cardiomyopathy
duchenne muscular dystrophy
genomic instability
mdx mouse
Journal
International journal of molecular sciences
ISSN: 1422-0067
Titre abrégé: Int J Mol Sci
Pays: Switzerland
ID NLM: 101092791
Informations de publication
Date de publication:
10 May 2021
10 May 2021
Historique:
received:
01
04
2021
revised:
30
04
2021
accepted:
07
05
2021
entrez:
2
6
2021
pubmed:
3
6
2021
medline:
16
6
2021
Statut:
epublish
Résumé
Duchenne muscular dystrophy (DMD) is a devastating condition shortening the lifespan of young men. DMD patients suffer from age-related dilated cardiomyopathy (DCM) that leads to heart failure. Several molecular mechanisms leading to cardiomyocyte death in DMD have been described. However, the pathological progression of DMD-associated DCM remains unclear. In skeletal muscle, a dramatic decrease in stem cells, so-called satellite cells, has been shown in DMD patients. Whether similar dysfunction occurs with cardiac muscle cardiovascular progenitor cells (CVPCs) in DMD remains to be explored. We hypothesized that the number of CVPCs decreases in the dystrophin-deficient heart with age and disease state, contributing to DCM progression. We used the dystrophin-deficient mouse model (
Identifiants
pubmed: 34068508
pii: ijms22095025
doi: 10.3390/ijms22095025
pmc: PMC8125982
pii:
doi:
Substances chimiques
Dystrophin
0
Proto-Oncogene Proteins c-kit
EC 2.7.10.1
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : Association Française contre les Myopathies
ID : 20225
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