Febuxostat ameliorates muscle degeneration and movement disorder of the dystrophin mutant model in Caenorhabditis elegans.
J Physiol Sci
; 73(1): 28, 2023 Nov 10.
Article
em En
| MEDLINE
| ID: mdl-37950170
ABSTRACT
Duchenne muscular dystrophy (DMD) is an inherited disorder with mutations in the dystrophin gene characterized by progressive muscle degeneration and weakness. Therapy such as administration of glucocorticoids, exon skipping of mutant genes and introduction of dystrophin mini-genes have been tried, but there is no radical therapy for DMD. In this study, we used C. elegans carrying mutations in the dys-1 gene as a model of DMD to examine the effects of febuxostat (FBX). We applied FBX to dys-1 mutant animals harboring a marker for muscle nuclei and mitochondria, and found that FBX ameliorates the muscle loss. We next used a severer model dys-1; unc-22 double mutant and found the dys-1 mutation causes a weakened muscle contraction. We applied FBX and other compounds to the double mutant animals and assayed the movement. We found that the administration of FBX in combination of uric acid has the best effects on the DMD model.
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Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Proteínas de Caenorhabditis elegans
/
Transtornos dos Movimentos
Limite:
Animals
Idioma:
En
Revista:
J Physiol Sci
Assunto da revista:
FISIOLOGIA
/
TRAUMATOLOGIA
Ano de publicação:
2023
Tipo de documento:
Article
País de afiliação:
Japão