Background: Coenzyme Q10 (CoQ10) deficiency is a rare but often treatable condition. In view of the encephalopathic presentation of this condition the aim of this project was to establish a neuronal cell model of CoQ10 deficiency in cultured neuroblastoma, SHSY-5Y cells using paraaminobenzoic acid (PABA) treatment. Methods: Treatment of SHSY-5Y cells with 1 mM PABA induced a maximal 54% (46% residual CoQ10; p<0.01) decrease in celluar CoQ10 status compared to control levels.