Document Detail

Complex I deficiency in Persian multiple sclerosis patients.
MedLine Citation:
PMID:  16413582     Owner:  NLM     Status:  MEDLINE    
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.
Hassan H Kumleh; Gholam H Riazi; Massoud Houshmand; Mohammad H Sanati; Kourosh Gharagozli; Mehdi Shafa
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2006-01-18
Journal Detail:
Title:  Journal of the neurological sciences     Volume:  243     ISSN:  0022-510X     ISO Abbreviation:  J. Neurol. Sci.     Publication Date:  2006 Apr 
Date Detail:
Created Date:  2006-03-22     Completed Date:  2006-06-01     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  0375403     Medline TA:  J Neurol Sci     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  65-9     Citation Subset:  IM    
Institute of Biochemistry and Biophysics, University of Tehran, Tehran, Iran.
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MeSH Terms
Central Nervous System / metabolism*,  physiopathology
Electron Transport Complex I / deficiency*,  genetics
Energy Metabolism / genetics
Infectious Disease Transmission, Vertical
Mitochondria / genetics,  metabolism*
Mitochondrial Diseases / complications,  genetics,  metabolism*
Multiple Sclerosis / genetics,  metabolism*,  physiopathology
Muscle, Skeletal / metabolism,  physiopathology
NADH, NADPH Oxidoreductases / analysis,  genetics,  metabolism
Nerve Degeneration / genetics,  metabolism,  physiopathology
Neurons / metabolism,  pathology
Reg. No./Substance:
EC 1.6.-/NADH, NADPH Oxidoreductases; EC Transport Complex I; EC 1.6.99.-/ferricyanide reductase

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