Indeed, several mouse or cell models for IEM, in which acyl-CoAs accumulate, display high levels of lysine acylation, including models for propionyl-CoA carboxylase (PCC) deficiency (OMIM 606054), glutaryl-CoA dehydrogenase (GCDH) deficiency (OMIM 231670), malonyl-CoA decarboxylase (MCD) deficiency (OMIM 248360) and short-chain acyl-CoA dehydrogenase (SCAD) deficiency (OMIM 201470) (Pougovkina et al 2014b; Tan et al 2014; Colak et al 2015). The gene discussed is GCDH; the disease is hyperinsulinemic hypoglycemia, familial, 4.