CSPG4 and congenital muscular dystrophy due to LMNA mutation: The expression of CSPG4 in muscle cells together with the fact that it is upregulated in Duchenne muscular dystrophy, and downregulated in merosin-deficient congenital muscular dystrophy (MDC1A) muscles, respectively (Petrini et al., 2003), suggest that Perd function in myofibrillogenesis might be conserved.