In the current study, we identified Atp6v0d1 as a crucial regulator of adipogenesis, and showed that loss of adipose Atp6v0d1 in mice resulted in a gradual loss of adipose tissues accompanied by systemic insulin resistance, hypercholesterolemia and hepatosteatosis, which are key features of generalized lipodystrophy 38, 39. This evidence concerns the gene ATP6V0D1 and familial hypercholesterolemia.