However, the conclusion of endogenous activation of compensatory mechanisms in muscle of MDC1A-patients is further supported by the increase of C4b-binding protein alpha chain belonging to the complement system that deposits its activation products on innervated motor end-plates in ALS-patients (35).These combined findings in turn underline the significance our proteomic profiling data aiding the identification of marker proteins with pathophysiological relevance as well as impacts for attempts to develop new treatment strategies for MDC1A. This evidence concerns the gene LAMA2 and amyotrophic lateral sclerosis.