Considering one of the major secretors of ECM proteins is the fibroblast cell, and matriglycans are differentially downregulated in human patients suffering different types of dystroglycanopathies, we decided to compare the proteome of cultured primary fibroblast cells from a WWS patient carrying an ISPD mutation and LGMD patients carrying FKRP mutations. The gene discussed is FKRP; the disease is neuromuscular disease caused by qualitative or quantitative defects of alpha-dystroglycan.