The Open Spine Journal

2009, 1 : 5-8
Published online 2009 March 17. DOI: 10.2174/1876532700901010005
Publisher ID: TOSPINEJ-1-5

Mitochondrial Superoxide Dismutase Activity in Amyotrophic Lateral Sclerosis

Pilar Larrodé , Pedro Iñarrea , Nobuhide Ogihara , Hiroki Hirabayashi , José L. Capablo , Cristina Iñiguez , José R. Ara , Jesús Martin and Enrique Mostacero1
Neurology Department, Lozano-Blesa Clínical Hospital, San Juan Bosco Av, nº 15, 50,009 Zaragoza, Spain

ABSTRACT

Background:

Evidence suggests that mitochondrial dysfunction and oxidative stress may be involved in the pathogenesis of Amyotrophic Lateral Sclerosis (ALS). Some studies show the presence of altered anti-oxidative defence enzyme activity in the blood of ALS patients. It has also been demonstrated that a superoxide-dismutase-1 (SOD1) enzyme fraction is located in the mitochondria.

Objective:

To Study the correlation between SOD1 activity and clinical data in ALS-patients. Patients and Methods: SOD1, SOD2 and aconitase activity was analysed in mitochondria of platelets from nine sporadicALS (SALS) patients and nine healthy-controls. The biochemical data was compared and correlated with clinical data from the ALS-patients.

Results:

Mitochondrial SOD1 activity was lower in ALS patients than in healthy controls, and much lower in more serious cases of ALS associated with respiratory insufficiency, weight-loss, low scores on the ALSFRS scale and the elderly.

Conclusions:

Conclusion: Mitochondrial SOD1 activity may be involved in the clinical evolution of SALS.

Keywords:

Amyotrophic lateral sclerosis,, antioxidant enzymes, mitochondrial dysfunction, oxidative stress,, superoxidedismutase..