GsMTx4-D provides protection to the D2.mdx mouse

Neuromuscular Disorders : NMD
Christopher W WardThomas M Suchyna

Abstract

Duchenne muscular dystrophy is a life-limiting muscle disease that has no current effective therapy. Despite mounting evidence that dysregulation of mechanosensitive ion channels is a significant contributor to dystrophy pathogenesis, effective pharmacologic strategies targeting these channels are lacking. GsMTx4, and its enantiomer GsMTx4-D, are peptide inhibitors of mechanosensitive channels with identical activity. In previous studies, acute in vitro application of GsMTx4 to dystrophic murine muscle effectively reduced the excess MSC dependent calcium influx linked to contraction-induced muscle damage. Here we sought to determine if in vivo treatment with GsMTx4-D proffered benefit in the D2.mdx mouse. GsMTx4-D showed a 1-week half-life when administered by subcutaneous injection over four weeks. Informed by these results, D2.mdx mice were then treated by a subcutaneous injection regimen of GsMTx4-D for six weeks followed by determination of muscle mass, muscle susceptibility to eccentric contraction injury and multiple histological indicators of disease progression. The mice showed a reduction in the loss of muscle mass and a decrease in susceptibility to contraction induced injury. These protective effects were realized wi...Continue Reading

Citations

Oct 10, 2019·Glia·María Velasco-EstevezGraham K Sheridan
Aug 21, 2020·JCI Insight·Reyhan WestbrookPeter M Abadir
Mar 7, 2021·International Journal of Molecular Sciences·Shashank ShekharGeorge W Booz
Aug 27, 2021·JCI Insight·Janelle Geist HausermanAikaterini Kontrogianni-Konstantopoulos

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