Here, we found that Atp6v0d1AKO mice display a similar developmental trajectory of cardiomyopathy to that observed in Bscl2-/- mice, the most widely used animal model for studying lipodystrophy-related cardiomyopathy recapitulating human type 2 Berardinelli-Seip congenital lipodystrophy (BSCL2) disease, which progresses from compensated cardiac hypertrophy to heart failure 40. Here, BSCL2 is linked to heart failure.