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. The gene discussed is ATP6V0D1; the disease is generalized lipodystrophy.