Direct sequencing of the BAG3 coding sequence and splice sites showed four different mutations in five DCM families: known missense Glu455Lys mutation in 2 families (DCM-16 and DCM-71), two novel small deletions predicted to cause frameshifts: Gln353ArgfsX10 (c.1055delC) (DCM-18), Gly379AlafsX45 (c.1135delG) (DCM-15), and a novel Tyr451X (c.1353C>A) mutation predicted to insert a premature stop codon (DCM-84). Here, BAG3 is linked to familial dilated cardiomyopathy.