Effects of Glucocorticoids in Murine Models of Duchenne and Limb-Girdle Muscular Dystrophy

Abstract

In vivo testing of glucocorticoid steroids in dystrophic mice offers important insights in benefits and risks of those drugs in the pathological context of muscular dystrophy. Frequency of dosing changes the spectrum of glucocorticoid effects on muscle and metabolic homeostasis. Here, we describe a combination of non-invasive and invasive methods to quantitatively discriminate the specific effects of intermittent (once-weekly) versus mainstay (once-daily) regimens on muscle fibrosis, muscle function, and metabolic homeostasis in murine models of Duchenne and limb-girdle muscular dystrophies.

Keywords: Glucocorticoid steroids; Glycemia; Grip strength; Hydroxyproline; In situ force analysis; Intermittent dosing; Sgcg mice; Treadmill assay; mdx mice.