DES and Desminopathy: In fact, both muscular laminopathies and desminopathies have defects in the secretome, with activation of an inflammatory and profibrotic response involving TGFbeta 2 and other cytokine increase, as in LMNA-RD [145], or activation of the complement and osteopontin overexpression in the myocardium of mice featuring desmin-related cardiomyopathy [90,91].