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Opa1 overexpression ameliorates the clinical phenotype of two mitochondrial disease mouse models

Date:
June 4, 2015
Source:
MRC Mitochondrial Biology Unit
Summary:
Scientists identify Opa1 as a potential therapeutic target to combat mitochondrial disease. OPA1 is a protein of the inner mitochondrial membrane, which shapes mitochondrial cristae, the structures where the respiratory complexes are located, thus regulating the efficiency of the respiratory chain.
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Mitochondrial diseases are highly heterogeneous disorders characterized by faulty oxidative phosphorylation. OPA1 is a protein of the inner mitochondrial membrane, which shapes mitochondrial cristae, the structures where the respiratory complexes are located, thus regulating the efficiency of the respiratory chain.

Civiletto et al. have exploited this concept to investigate whether moderate Opa1 overexpression in tissues could ameliorate the clinical and biochemical phenotype of two mouse models of severe mitochondrial disease, characterized by deficiency in respiratory complex I and IV, respectively. These biochemical defects lead to reduced motor and coordination skill, reduced lifespan and reduced activity of the respective respiratory complexes. The mice with increased OPA1 levels showed a marked improvement in both clinical and biochemical defects of the two mouse models. These effects were related to the correction of the mitochondrial ultrastructure and stabilization of the respiratory complexes and supercomplexes. By identifying OPA1 as a new potential target, this study opens new avenues towards evidence-based effective therapy for mitochondrial disorders.


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Materials provided by MRC Mitochondrial Biology Unit. Note: Content may be edited for style and length.


Journal Reference:

  1. Gabriele Civiletto, Tatiana Varanita, Raffaele Cerutti, Tatiana Gorletta, Serena Barbaro, Silvia Marchet, Costanza Lamperti, Carlo Viscomi, Luca Scorrano, Massimo Zeviani. Opa1 Overexpression Ameliorates the Phenotype of Two Mitochondrial Disease Mouse Models. Cell Metabolism, 2015; 21 (6): 845 DOI: 10.1016/j.cmet.2015.04.016

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MRC Mitochondrial Biology Unit. "Opa1 overexpression ameliorates the clinical phenotype of two mitochondrial disease mouse models." ScienceDaily. ScienceDaily, 4 June 2015. <www.sciencedaily.com/releases/2015/06/150604141626.htm>.
MRC Mitochondrial Biology Unit. (2015, June 4). Opa1 overexpression ameliorates the clinical phenotype of two mitochondrial disease mouse models. ScienceDaily. Retrieved May 29, 2017 from www.sciencedaily.com/releases/2015/06/150604141626.htm
MRC Mitochondrial Biology Unit. "Opa1 overexpression ameliorates the clinical phenotype of two mitochondrial disease mouse models." ScienceDaily. www.sciencedaily.com/releases/2015/06/150604141626.htm (accessed May 29, 2017).

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