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5) [ 20 ] ; type III 3-MGC aciduria (Costeff optic atrophy syndrome) (Mendelian Inheritance in Man database *258501) was described in patients of Iraqi-Jewish origin with bilateral optic atrophy accompanied in some by movement disorders and caused by mutations in the OPA3 gene [ 21 22 ] ; and type IV 3-MGC aciduria (Mendelian Inheritance in Man database *250951) presents with moderate to severe neurological disease, cardiac, ophthalmic, hepatic, and renal symptoms and it has been associated with mitochondrial disorders (Pearson syndrome, mitochondrial DNA depletion) or multiple respiratory chain defects [ 23 24 25 26 ] . This group does not have a defined primary biochemical or molecular defect.