Cardiovascular progenitor cells and tissue plasticity are reduced in a myocardium affected by Becker muscular dystrophy.
Becker muscular dystrophy
C-kit
Cardiomyopathy
Cardiovascular progenitor cells
Dystrophin
Heart failure
Journal
Orphanet journal of rare diseases
ISSN: 1750-1172
Titre abrégé: Orphanet J Rare Dis
Pays: England
ID NLM: 101266602
Informations de publication
Date de publication:
05 03 2020
05 03 2020
Historique:
received:
23
07
2019
accepted:
19
11
2019
entrez:
7
3
2020
pubmed:
7
3
2020
medline:
22
6
2021
Statut:
epublish
Résumé
We describe the association of Becker muscular dystrophy (BMD) derived heart failure with the impairment of tissue homeostasis and remodeling capabilities of the affected heart tissue. We report that BMD heart failure is associated with a significantly decreased number of cardiovascular progenitor cells, reduced cardiac fibroblast migration, and ex vivo survival. Becker muscular dystrophy belongs to a class of genetically inherited dystrophin deficiencies. It affects male patients and results in progressive skeletal muscle degeneration and dilated cardiomyopathy leading to heart failure. It is a relatively mild form of dystrophin deficiency, which allows patients to be on a heart transplant list. In this unique situation, the explanted heart is a rare opportunity to study the degenerative process of dystrophin-deficient cardiac tissue. Heart tissue was excised, dissociated, and analyzed. The fractional content of c-kit We report significantly decreased CVPCs (c-kit Our findings associate genetically derived heart failure in a dystrophin-deficient patient with decreased c-kit
Sections du résumé
We describe the association of Becker muscular dystrophy (BMD) derived heart failure with the impairment of tissue homeostasis and remodeling capabilities of the affected heart tissue. We report that BMD heart failure is associated with a significantly decreased number of cardiovascular progenitor cells, reduced cardiac fibroblast migration, and ex vivo survival.
BACKGROUND
Becker muscular dystrophy belongs to a class of genetically inherited dystrophin deficiencies. It affects male patients and results in progressive skeletal muscle degeneration and dilated cardiomyopathy leading to heart failure. It is a relatively mild form of dystrophin deficiency, which allows patients to be on a heart transplant list. In this unique situation, the explanted heart is a rare opportunity to study the degenerative process of dystrophin-deficient cardiac tissue. Heart tissue was excised, dissociated, and analyzed. The fractional content of c-kit
RESULTS
We report significantly decreased CVPCs (c-kit
CONCLUSIONS
Our findings associate genetically derived heart failure in a dystrophin-deficient patient with decreased c-kit
Identifiants
pubmed: 32138751
doi: 10.1186/s13023-019-1257-4
pii: 10.1186/s13023-019-1257-4
pmc: PMC7057505
doi:
Substances chimiques
Dystrophin
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
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