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Originally published as Genetics Published Articles Ahead of Print on April 15, 2007.

Genetics, Vol. 176, 937-946, June 2007, Copyright © 2007
doi:10.1534/genetics.107.071688

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Mutations in Cytochrome c Oxidase Subunit VIa Cause Neurodegeneration and Motor Dysfunction in Drosophila

Wensheng Liu*,1, Radhakrishnan Gnanasambandam{dagger}, Jeffery Benjamin{dagger}, Gunisha Kaur*,2, Patricia B. Getman*, Alan J. Siegel{dagger}, Randall D. Shortridge{dagger} and Satpal Singh*,3

* Department of Pharmacology and Toxicology, State University of New York, Buffalo, New York 14214 and {dagger} Department of Biological Sciences, State University of New York, Buffalo, New York 14260

3 Corresponding author: Department of Pharmacology and Toxicology, 102 Farber Hall, State University of New York, Buffalo, NY 14214-3000.
E-mail: singhs{at}buffalo.edu

Mitochondrial dysfunction is involved in many neurodegenerative disorders in humans. Here we report mutations in a gene (designated levy) that codes for subunit VIa of cytochrome c oxidase (COX). The mutations were identified by the phenotype of temperature-induced paralysis and showed the additional phenotypes of decreased COX activity, age-dependent bang-induced paralysis, progressive neurodegeneration, and reduced life span. Germ-line transformation using the levy+ gene rescued the mutant flies from all phenotypes including neurodegeneration. The data from levy mutants reveal a COX-mediated pathway in Drosophila, disruption of which leads to mitochondrial encephalomyopathic effects including neurodegeneration, motor dysfunction, and premature death. The data present the first case of a mutation in a nuclear-encoded structural subunit of COX that causes mitochondrial encephalomyopathy rather than lethality, whereas several previous attempts to identify such mutations have not been successful. The levy mutants provide a genetic model to understand the mechanisms underlying COX-mediated mitochondrial encephalomyopathies and to explore possible therapeutic interventions.


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