Duchenne muscular dystrophy is primarily characterized by progressive muscle
wasting due to deficiency in the membrane cytoskeletal protein dystrophin but
is also associated with body-wide cellular disturbances in a variety of non-muscle
tissues. In this study, we have focused on the comparative proteomic analysis of
the spleen and established considerable changes in this crucial secondary
lymphoid organ from the genetic mdx-4cv mouse model of dystrophinopathy.
An apparent short isoform of dystrophin and associated glycoproteins were identified in spleen by mass spectrometry but appear not be affected in muscular dystrophy. In contrast, the mdx-4cv spleen showed significant proteome-wide
changes in other protein species that are involved in metabolism, signaling, and
cellular architecture. Since the spleen plays a key role in the immune response,
these proteomic alterations may reflect pathophysiological cross talk between
the lymphoid system and dystrophic muscles, which are affected by both fiber
degeneration and inflammation