Nintedanib Reduces Muscle Fibrosis and Improves Muscle Function of the Alpha-Sarcoglycan-Deficient Mice.
Sgca
fibrosis
mice
muscular dystrophy
nintedanib
sarcoglycanopathy
Journal
Biomedicines
ISSN: 2227-9059
Titre abrégé: Biomedicines
Pays: Switzerland
ID NLM: 101691304
Informations de publication
Date de publication:
19 Oct 2022
19 Oct 2022
Historique:
received:
09
09
2022
revised:
09
10
2022
accepted:
15
10
2022
entrez:
27
10
2022
pubmed:
28
10
2022
medline:
28
10
2022
Statut:
epublish
Résumé
Sarcoglycanopathies are a group of recessive limb-girdle muscular dystrophies, characterized by progressive muscle weakness. Sarcoglycan deficiency produces instability of the sarcolemma during muscle contraction, leading to continuous muscle fiber injury eventually producing fiber loss and replacement by fibro-adipose tissue. Therapeutic strategies aiming to reduce fibro-adipose expansion could be effective in muscular dystrophies. We report the positive effect of nintedanib in a murine model of alpha-sarcoglycanopathy. We treated 14 Sgca
Identifiants
pubmed: 36289891
pii: biomedicines10102629
doi: 10.3390/biomedicines10102629
pmc: PMC9599168
pii:
doi:
Types de publication
Journal Article
Langues
eng
Subventions
Organisme : Medical Research Council
ID : MR/W019086/1
Pays : United Kingdom
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