Human idiopathic OAT, which shares several characteristics with the MLXKO phenotype, is typically associated with a variety of metabolism-related pathologies, including metabolic syndrome, diabetes, obesity, and inflammation [61], all of which have been reported to be linked with dysregulation of MLX’s dimerization partners MondoA and/or ChREBP (reviewed in [62,63]). Here, MLXIP is linked to obesity due to melanocortin 4 receptor deficiency.