Login to MyKarger

New to MyKarger? Click here to sign up.

Login with Facebook

Forgot Password? Reset your password

Authors, Editors, Reviewers

For Manuscript Submission, Check or Review Login please go to Submission Websites List.

Submission Websites List

Institutional Login (Shibboleth)

For the academic login, please select your country in the dropdown list. You will be redirected to verify your credentials.

Table of Contents
Vol. 1, No. 6, 2004
Issue release date: May 2005
Section title: Review
Neurodegenerative Dis 2004;1:245–254

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
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
email 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

Do you have an account?

Login Information

Contact Information

I have read the Karger Terms and Conditions and agree.


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

Copyright / Drug Dosage / Disclaimer

Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.