title: Complex I deficiency in Persian multiple sclerosis patients.
abstract: 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 on intact mitochondria isolated from fresh skeletal muscle in 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 in mitochondrial disorders to those of MS. This study suggested that a biochemical defect in complex I activity may be involved in pathogenesis of MS.
authors.affiliation: Institute of Biochemistry and Biophysics, University of Tehran, Tehran, Iran.
authors.forename: Hassan H
authors.initials: HH
authors.lastname: Kumleh
authors.forename: Gholam H
authors.initials: GH
authors.lastname: Riazi
authors.forename: Massoud
authors.initials: M
authors.lastname: Houshmand
authors.forename: Mohammad H
authors.initials: MH
authors.lastname: Sanati
authors.forename: Kourosh
authors.initials: K
authors.lastname: Gharagozli
authors.forename: Mehdi
authors.initials: M
authors.lastname: Shafa
mesh_terms: D000328:Adult; D001706:Biopsy; D002490:Central Nervous System / Q000378:metabolism* / Q000503:physiopathology; D042967:Electron Transport Complex I / Q000172:deficiency* / Q000235:genetics; D004734:Energy Metabolism / Q000235:genetics; D005260:Female; D006801:Humans; D018445:Infectious Disease Transmission, Vertical; D008297:Male; D008928:Mitochondria / Q000235:genetics / Q000378:metabolism*; D028361:Mitochondrial Diseases / Q000150:complications / Q000235:genetics / Q000378:metabolism*; D009103:Multiple Sclerosis / Q000235:genetics / Q000378:metabolism* / Q000503:physiopathology; D018482:Muscle, Skeletal / Q000378:metabolism / Q000503:physiopathology; D009247:NADH, NADPH Oxidoreductases / Q000032:analysis / Q000235:genetics / Q000378:metabolism; D009410:Nerve Degeneration / Q000235:genetics / Q000378:metabolism / Q000503:physiopathology; D009474:Neurons / Q000378:metabolism / Q000473:pathology; D013053:Spectrophotometry
chemical_list: D009247:NADH, NADPH Oxidoreductases; C014227:ferricyanide reductase; D042967:Electron Transport Complex I