Microbial peptide de-coppers mitochondria: implications for Wilson disease

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-...

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Bibliographic Details
Published inThe Journal of clinical investigation Vol. 126; no. 7; pp. 2412 - 2414
Main Author Kaler, Stephen G.
Format Journal Article
LanguageEnglish
Published United States American Society for Clinical Investigation 01.07.2016
Subjects
Adenosine Triphosphatases - chemistry
Animals
Binding sites
Biomedical research
Brain research
Cation Transport Proteins - chemistry
Clinical trials
Copper
Copper in the body
Copper-Transporting ATPases
Development and progression
Health aspects
Hepatolenticular Degeneration
Humans
Hypotheses
Liver
Liver diseases
Membrane separation
Metabolic disorders
Mitochondria
Peptides
Permeability
Properties
Rats
Rodents
Wilson's disease
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Summary: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.
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ISSN:0021-9738
1558-8238
DOI:10.1172/JCI88617