Ixazomib, an oral proteasome inhibitor, exhibits potential effect in dystrophin-deficient mdx mice.
duchenne muscular dystrophy
dystrophin
ixazomib
mdx mice
proteasome inhibitor
Journal
International journal of experimental pathology
ISSN: 1365-2613
Titre abrégé: Int J Exp Pathol
Pays: England
ID NLM: 9014042
Informations de publication
Date de publication:
02 2021
02 2021
Historique:
received:
29
06
2020
revised:
31
10
2020
accepted:
04
11
2020
pubmed:
10
12
2020
medline:
9
10
2021
entrez:
9
12
2020
Statut:
ppublish
Résumé
Dystrophin deficiency makes the sarcolemma fragile and susceptible to degeneration in Duchenne muscular dystrophy. The proteasome is a multimeric protease complex and is central to the regulation of cellular proteins. Previous studies have shown that proteasome inhibition improved pathological changes in mdx mice. Ixazomib is the first oral proteasome inhibitor used as a therapy in multiple myeloma. This study investigated the effects of ixazomib on the dystrophic muscle of mdx mice. MDX mice were treated with ixazomib (7.5 mg/kg/wk by gavage) or 0.2 mL of saline for 12 weeks. The Kondziela test was performed to measure muscle strength. The tibialis anterior (TA) and diaphragm (DIA) muscles were used for morphological analysis, and blood samples were collected for biochemical measurement. We observed maintenance of the muscle strength in the animals treated with ixazomib. Treatment with ixazomib had no toxic effect on the mdx mouse. The morphological analysis showed a reduction in the inflammatory area and fibres with central nuclei in the TA and DIA muscles and an increase in the number of fibres with a diameter of 20 µm
Identifiants
pubmed: 33296126
doi: 10.1111/iep.12383
pmc: PMC7839951
doi:
Substances chimiques
Boron Compounds
0
Dystrophin
0
Protease Inhibitors
0
Utrophin
0
ixazomib
71050168A2
Glycine
TE7660XO1C
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
11-21Informations de copyright
© 2020 Company of the International Journal of Experimental Pathology (CIJEP).
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