Journal article

BGP-15 Improves Aspects of the Dystrophic Pathology in mdx and dko Mice with Differing Efficacies in Heart and Skeletal Muscle

Tahnee L Kennedy, Kristy Swiderski, Kate T Murphy, Stefan M Gehrig, Claire L Curl, Chanchal Chandramouli, Mark A Febbraio, Lea MD Delbridge, Rene Koopman, Gordon S Lynch



Duchenne muscular dystrophy is a severe and progressive striated muscle wasting disorder that leads to premature death from respiratory and/or cardiac failure. We have previously shown that treatment of young dystrophic mdx and dystrophin/utrophin null (dko) mice with BGP-15, a coinducer of heat shock protein 72, ameliorated the dystrophic pathology. We therefore tested the hypothesis that later-stage BGP-15 treatment would similarly benefit older mdx and dko mice when the dystrophic pathology was already well established. Later stage treatment of mdx or dko mice with BGP-15 did not improve maximal force of tibialis anterior (TA) muscles (in situ) or diaphragm muscle strips (in vitro). Howev..

View full abstract


Awarded by Muscular Dystrophy Association USA research grant

Awarded by National Health and Medical Research Council of Australia (NHMRC)

Funding Acknowledgements

Supported by the Muscular Dystrophy Association USA research grant MDA255253 (G.S.L.) and the National Health and Medical Research Council of Australia (NHMRC) research grant APP106546 (G.S.L.); the Heart Foundation (Australia) initially by an Australian Postgraduate Award (T.L.K.) and then a Postgraduate Scholarship (T.L.K); NHMRC Early Career Fellowship (K.S.); NHMRC Career Development Fellowship (K.T.M.); and NHMRC Senior Principal Research Fellowship (M.A.F.).