AGPAT2 and Berardinelli-Seip congenital lipodystrophy: Among the AGPAT isoforms, AGPAT2 stands out since null mutations of AGPAT2 are associated with Berardinelli-Seip congenital lipodystrophy type 1 (BSCL1)/congenital generalized lipodystrophy, type 1 (CGL1), which is characterized by a near complete loss of adipose tissue, early onset of insulin resistance, diabetes, hypertriglyceridemia, and hepatic steatosis9.