Differential Effects of Halofuginone Enantiomers on Muscle Fibrosis and Histopathology in Duchenne Muscular Dystrophy
نویسندگان
چکیده
Progressive loss of muscle and function is associated with significant fibrosis in Duchenne muscular dystrophy (DMD) patients. Halofuginone, an analog febrifugine, prevents various animal models, including those dystrophies. Effects (+)/(?)-halofuginone enantiomers on motor coordination diaphragm histopathology mdx mice, the mouse model for DMD, were examined. Four-week-old male mice treated racemic halofuginone, or its separate enantiomers, 10 weeks. Controls saline. Racemic halofuginone-treated demonstrated better balance than controls. However, (+)-halofuginone surpassed form’s effect. No effect was observed (?)-halofuginone, which behaved like control. A reduction collagen content degenerative areas, increase utrophin levels diaphragms halofuginone. Again, more effective form, whereas (?)-halofuginone had no Both increased myofiber diameters, (?)-halofuginone. effects any compounds tested in-vitro cell viability assay. These results, demonstrating a differential halofuginone superiority (+)-halofuginone, are great importance future use as DMD antifibrotic therapy.
منابع مشابه
Focusing on fibrosis: halofuginone-induced functional improvement in the mdx mouse model of Duchenne muscular dystrophy.
Corresponding Author: Linda K McLoon, Ph.D. Department of Ophthalmology University Of Minnesota Room 374 LRB, 2001 6 Street SE Minneapolis, MN 55455 Tel: 612-626-0777 Fax: 612-626-0781 Email: [email protected] Running title: Focusing on Fibrosis Submitted as an Editorial Focus to American Journal of Physiology: Heart and Circulatory Physiology Articles in PresS. Am J Physiol Heart Circ Physiol (...
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ژورنال
عنوان ژورنال: International Journal of Molecular Sciences
سال: 2021
ISSN: ['1661-6596', '1422-0067']
DOI: https://doi.org/10.3390/ijms22137063