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Enzyme engineering to fight mitochondrial diseases

Nature Metabolism volume 2, pages 215–216 (2020)Cite this article

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Inherited deficiencies in oxidative phosphorylation cause severe mitochondrial disease. A recent study in Nature Biotechnology demonstrates that an engineered bifunctional enzyme can rescue the biochemical consequences of mitochondrial dysfunction.

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Fig. 1: Extracellular lactate-to-pyruvate conversion by LOXCAT restores the intracellular NADH/NAD+ ratio.

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Authors and Affiliations

  1. Laboratory of Genetic Metabolic Diseases, Amsterdam Gastroenterology and Metabolism, Amsterdam Cardiovascular Sciences, Amsterdam UMC, University of Amsterdam, Amsterdam, the Netherlands

    Rubén Zapata-Pérez & Riekelt H. Houtkooper

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  1. Rubén Zapata-Pérez
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  2. Riekelt H. Houtkooper
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Correspondence to Riekelt H. Houtkooper.

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The authors declare no competing interests.

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Zapata-Pérez, R., Houtkooper, R.H. Enzyme engineering to fight mitochondrial diseases. Nat Metab 2, 215–216 (2020). https://doi.org/10.1038/s42255-020-0177-x

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