Article
Molecular mechanism of sphingosine-1-phosphate action in Duchenne muscular dystrophy.
Disease models & mechanisms - 1 Jan 2014
Nguyen-Tran Diem-Hang, Hait Nitai C, Sperber Henrik, Qi Junlin, Fischer Karin, Ieronimakis Nick, Pantoja Mario, Hays Aislinn, Allegood Jeremy, Reyes Morayma, Spiegel Sarah, Ruohola-Baker Hannele
Abstract excerpt
Duchenne muscular dystrophy (DMD) is a lethal muscle-wasting disease. Studies in Drosophila showed that genetic increase of the levels of the bioactive sphingolipid sphingosine-1-phosphate (S1P) or delivery of 2-acetyl-5-tetrahydroxybutyl imidazole (THI), an S1P lyase inhibitor, suppresses dystrophic muscle degeneration. In the dystrophic mouse (mdx), upregulation of S1P by THI increases regeneration and muscle...
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