Homozygous disruption of either BSCL2 or AGPAT2, encoding seipin and 1-acylglycerol-3-phosphate O-acyltransferase 2 (AGPAT2) respectively, causes a very severe congenital generalized lipodystrophy (CGL). This evidence concerns the gene BSCL2 and generalized lipodystrophy.