摘要：SummaryDuchenne 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 geneticmdx-4cvmouse 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, themdx-4cvspleen 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.Graphical AbstractDisplay OmittedHighlights•Biochemical and proteomic analyses have identified dystrophin isoform Dp71 in spleen•Normal Dp71 levels were found in the spleen of themdx-4cvmodel of dystrophinopathy•The enzymes TGM2 and MMP-9 are drastically increased inmdx-4cvspleen•Apolipoproteins ApoE and ApoB are greatly reduced inmdx-4cvspleenDisease; Pathophysiology; Proteomics
