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The mitochondrial brain: From mitochondrial genome to neurodegeneration

Lookup NU author(s): Dr Nichola Lax, Dr Daria Diodato, Emeritus Professor Doug Turnbull

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Abstract

Mitochondrial DNA mutations are an important cause of neurological disease. The clinical presentation is very varied in terms of age of onset and different neurological signs and symptoms. The clinical course varies considerably but in many patients there is a progressive decline, and in some evidence of marked neurodegeneration. Our understanding of the mechanisms involved is limited due in part to limited availability of animal models of disease. However, studies on human post-mortem brains, combined with clinical and radiological studies, are giving important insights into specific neuronal involvement. (C) 2009 Elsevier B.V. All rights reserved.

Publication metadata

Author(s): Turnbull HE, Lax NZ, Diodato D, Ansorge O, Turnbull DM

Publication type: Review

Publication status: Published

Journal: Biochimica et Biophysica Acta: Molecular Basis of Disease

Year: 2010

Volume: 1802

Issue: 1

Pages: 111-121

ISSN (print): 0925-4439

ISSN (electronic): 0006-3002

Publisher: Elsevier BV

URL: http://dx.doi.org/10.1016/j.bbadis.2009.07.010

DOI: 10.1016/j.bbadis.2009.07.010

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