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Modem medicine offers no cure for patients suffering from mitochondrial disorders.We therefore set out to develop a novel approach to treat mitochondrial disorders that are caused by deficiencies in enzymes activities using celldirected enzyme replacement therapy.As a proof-of-principle to this novel approach we have chosen the mitochondrial disorder lipoamide dehydrogenase (LAD) deficiency.LAD is the E3 subunit shared by all-ketoacid dehydrogenase complexes in the mitochondrial matrix, which are crucial for the metabolism of sugars and amino acids.