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Strategies for treating disorders of the mitochondrial genome

Lookup NU author(s): Dr Gunther Ross, Professor Robert Taylor, Emeritus Professor Doug Turnbull, Professor Robert Lightowlers


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Defects of the mitochondrial genome are a significant cause of disease. Patients suffer from a wide variety of clinical presentations, ranging from fatal infantile disease to mild muscle weakness. Most disorders, however, are characterized by inexorable progression: As mutations often cause defects in several components of the complexes that couple oxidative phosphorylation, this terminal state of oxidative metabolism cannot be readily bypassed by dietary means, leading to the search for novel therapies. In this article, we present the theory behind several concepts and report progress. We also discuss some of the recent difficulties encountered in the progress towards an antigenome approach to treating mtDNA disorders. (C) 2004 Elsevier B.V. All rights reserved.

Publication metadata

Author(s): Smith PM, Ross GF, Taylor RW, Turnbull DM, Lightowlers RN

Publication type: Conference Proceedings (inc. Abstract)

Publication status: Published

Conference Name: Biochimica et Biophysica Acta: Bioenergetics. 6th European Meeting on Mitochondrial Pathology (Euromit 6)

Year of Conference: 2004

Pages: 232-239

ISSN: 0005-2728

Publisher: Elsevier BV


DOI: 10.1016/j.bbabio.2004.09.003

Library holdings: Search Newcastle University Library for this item

ISBN: 00063002