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- Title
Microbial peptide de-coppers mitochondria: implications for Wilson disease.
- Authors
Kaler, Stephen G.
- 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.
- Subjects
HEPATOLENTICULAR degeneration; CHELATION therapy; COPPER metabolism disorders; MITOCHONDRIA; COPPER binding proteins; LIVER transplantation; ADENOSINE triphosphatase; ANIMALS; CARRIER proteins; COPPER; RATS; RESEARCH funding
- Publication
Journal of Clinical Investigation, 2016, Vol 126, Issue 7, p2412
- ISSN
0021-9738
- Publication type
journal article
- DOI
10.1172/JCI88617