Complex I deficiency in Persian multiple sclerosis patients

Multiple sclerosis (MS) is a demyelinating disease of the central nervous system characterized by the morphological hallmarks of inflammation, demyelination and axonal loss. Until now, little attention has been paid to the contribution of mitochondrial respiratory chain enzyme activities to MS. In this study, kinetic analysis of mitochondrial respiratory chain complex I enzyme (measured as NADH-ferricyanide reductase) was performed oil intact mitochondria isolated from fresh skeletal Muscle ill MS patients (n = 10) and control subjects (n = 11). Mitochondrial DNA common deletion and deletions were also tested in MS patients. Our findings showed that complex I activities were significantly reduced (P = 0.007) in patients compared with control. However, We could not find deletion in mtDNA of patients with MS. The presupposition of relationship between MS and mitochondrial disorders is due to predominant maternal transmission of MS in affected parent-child pairs, pathoaetiological role of respiratory chain dysfunction in multisystem disorders and important role of it in neurodegenerative disorders, a number of patients Such as LHON or other mtDNA abnormality with developed neurological symptoms indistinguishable From MS and similarity of clinical symptoms ill mitochondrial disorders to those of MS. This Study Suggested that a biochemical defect in complex I activity may be involved in pathogenesis of MS. (c) 2005 Elsevier B.V. All rights reserved.