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Affected individuals are typically characterised by lipoatrophic diabetes, insulin resistance and hepatic steatosis ( Agonists of the glucagon-like peptide-1 receptor (GLP-1R) deliver significant beneficial effects both in treating type 2 diabetes and obesity ( Reports of the effects of GLP-1R agonists in individuals with inherited lipodystrophies are currently limited to only 3 case studies, and these examine FPLD but not CGL patients ( Here we used a well validated Bscl2 -null (seipin knockout) mouse model of CGL type 2 (CGL2) to examine the effectiveness of a GLP-1R agonist, in treating lipoatrophic diabetes, hepatic steatosis and hyperphagia in CGL to inform the adoption of this class of drugs for individuals with lipodystrophy

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doi: 10.1056/NEJMoa1611925