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Usage Information

Microbial peptide de-coppers mitochondria: implications for Wilson disease
Stephen G. Kaler
Stephen G. Kaler
Published June 20, 2016
Citation Information: J Clin Invest. 2016;126(7):2412-2414. https://doi.org/10.1172/JCI88617.
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Commentary

Microbial peptide de-coppers mitochondria: implications for Wilson disease

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Abstract

The severe liver pathology of untreated Wilson disease (WD) is associated with massive copper overload caused by mutations in a liver-specific copper-transporting ATPase, ATP7B. While early, presymptomatic detection and chelation with conventional copper-binding molecules enables effective and life-saving treatment, liver transplantation is the sole option currently available for those with advanced disease. In this issue of the JCI, Lichtmannegger, Leitzinger, and colleagues delineate the therapeutic effect of methanobactin (MB), a potent bacterial copper-binding protein, at three late stages of disease in a WD rat model. Their results suggest that a formal clinical trial of MB in human subjects with severe hepatic pathology caused by WD would be rational.

Authors

Stephen G. Kaler

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