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Mitochondria in Amyotrophic Lateral Sclerosis: A Trigger and a Target

Dupuis L.a, b · Gonzalez de Aguilar J.-L.a · Oudart H.c · de Tapia M.a · Barbeito L.b · Loeffler J.-P.a

Author affiliations

aLaboratoire de Signalisations Moléculaires et Neurodégénérescence, U692 INSERM, Faculté de Médecine, Université Louis Pasteur, Strasbourg, France; bInstituto de Investigaciones Biologicas Clemente Estable, Montevideo, Uruguay; cCEPE, CNRS UPR9010, Strasbourg, France

Corresponding Author

Jean-Philippe Loeffler

Laboratoire de Signalisations Moléculaires et Neurodégénérescence

U692 INSERM, Université Louis Pasteur, Faculté de Médecine, 11 rue Humann

FR–67085 Strasbourg (France)

Tel. +33 390 243081, Fax +33 390 243065, E-Mail loeffler@neurochem.u-strasbg.fr

Related Articles for ""

Neurodegenerative Dis 2004;1:245–254

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Strong evidence shows that mitochondrial dysfunction is involved in amyotrophic lateral sclerosis (ALS), but despite the fact that mitochondria play a central role in excitotoxicity, oxidative stress and apoptosis, the intimate underlying mechanism linking mitochondrial defects to motor neuron degeneration in ALS still remains elusive. Morphological and functional abnormalities occur in mitochondria in ALS patients and related animal models, although their exact nature and extent are controversial. Recent studies postulate that the mislocalization in mitochondria of mutant forms of copper-zinc superoxide dismutase (SOD1), the only well-documented cause of familial ALS, may account for the toxic gain of function of the enzyme, and hence induce motor neuron death. On the other hand, mitochondrial dysfunction in ALS does not seem to be restricted only to motor neurons as it is also present in other tissues, particularly the skeletal muscle. The presence of this ‘systemic’ defect in energy metabolism associated with the disease is supported in skeletal muscle tissue by impaired mitochondrial respiration and overexpression of uncoupling protein 3. In addition, the lifespan of transgenic mutant SOD1 mice is increased by a highly energetic diet compensating both the metabolic defect and the motorneuronal function. In this review, we will focus on the mitochondrial dysfunction linked to ALS and the cause-and-effect relationships between mitochondria and the pathological mechanisms thought to be involved in the disease.

© 2004 S. Karger AG, Basel

Article / Publication Details

First-Page Preview
Abstract of Review

Received: September 27, 2004
Accepted: January 24, 2005
Published online: June 02, 2005
Issue release date: May 2005

Number of Print Pages: 10
Number of Figures: 3
Number of Tables: 0

ISSN: 1660-2854 (Print)
eISSN: 1660-2862 (Online)

For additional information: http://www.karger.com/NDD

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