Mutation-independent Proteomic Signatures of Pathological Progression in Murine Models of Duchenne Muscular Dystrophy
Tirsa L.E. van Westering, Henrik J. Johansson, Britt Hanson, Anna Coenen-Stass, Yulia Lomonosova, Jun Tanihata, Norio Motohashi, Toshifumi Yokota, Shin’ichi Takeda, Janne Lehtiö, Matthew J. A. Wood, Samir EL Andaloussi, Yoshitsugu Aoki, Thomas C. Roberts
Abstract
TNF, INFγ, NF-κB, SIRT1, AMPK, PGC-1α, PPARs, ILK, and AKT/PI3K). Upregulation of CAV3, MVP and PAK1 protein expression was validated in dystrophic muscle by Western blot. Furthermore, MVP was upregulated during, but not required for, the differentiation of C2C12 myoblasts suggesting that this protein may affect muscle regeneration. This study provides novel insights into mutation-independent proteomic signatures characteristic of the dystrophic phenotype and its progression with aging.
Topics & Concepts
Duchenne muscular dystrophyPathologicalDystrophinMuscular dystrophyMutationMedicineBiologyPathologyGeneticsInternal medicineGeneMuscle Physiology and DisordersGenetic Neurodegenerative DiseasesCardiomyopathy and Myosin Studies