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Bioluminescent mice to monitor progression of muscular dystrophy

Mice with inducible luciferase expression in muscle stem cells could help identify new therapeutic candidates to treat muscular dystrophies. In the mice, muscle injury led to increased luciferase expression compared with baseline, and muscle recovery led to a return to baseline luciferase expression. In a mouse model for limb-girdle muscular dystrophy 2B that also had inducible luciferase expression in muscle stem cells, disease progression could be monitored via luciferase expression, which also correlated with the expression of conventional disease markers. Next steps could include using the model to monitor the effect of therapeutic candidates on muscular dystrophy progression.

SciBX 6(19); doi:10.1038/scibx.2013.475
Published online May 16, 2013

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Maguire, K.K. et al. J. Clin. Invest.; published online April 24, 2013;
doi:10.1172/JCI68458
Contact: Thomas A. Rando, Stanford University, Stanford, Calif.
e-mail:
rando@stanford.edu