Rapid restitution of contractile dysfunction by synthetic copolymers in dystrophin-deficient single live skeletal muscle fibers.
Calcium handling
Contractile function
Duchenne muscular dystrophy
Dystrophin
Membrane stabilizing copolymer
Skeletal muscle
Journal
Skeletal muscle
ISSN: 2044-5040
Titre abrégé: Skelet Muscle
Pays: England
ID NLM: 101561193
Informations de publication
Date de publication:
19 05 2023
19 05 2023
Historique:
received:
08
12
2022
accepted:
05
05
2023
medline:
22
5
2023
pubmed:
20
5
2023
entrez:
19
5
2023
Statut:
epublish
Résumé
Duchenne muscular dystrophy (DMD) is caused by the lack of dystrophin, a cytoskeletal protein essential for the preservation of the structural integrity of the muscle cell membrane. DMD patients develop severe skeletal muscle weakness, degeneration, and early death. We tested here amphiphilic synthetic membrane stabilizers in mdx skeletal muscle fibers (flexor digitorum brevis; FDB) to determine their effectiveness in restoring contractile function in dystrophin-deficient live skeletal muscle fibers. After isolating FDB fibers via enzymatic digestion and trituration from thirty-three adult male mice (9 C57BL10, 24 mdx), these were plated on a laminin-coated coverslip and treated with poloxamer 188 (P188; PEO
Identifiants
pubmed: 37208786
doi: 10.1186/s13395-023-00318-y
pii: 10.1186/s13395-023-00318-y
pmc: PMC10197332
doi:
Substances chimiques
Dystrophin
0
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
9Informations de copyright
© 2023. The Author(s).
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