A microbial peptide to rescue severe and fulminant Wilson disease?

Wilson disease is characterized by massive copper overload caused by a mutation of the liver-specific copper-transporting ATPase, ATP7B. Presently, liver transplantation is the only treatment available to patients with advanced or acute liver disease. In this paper, the authors describe the therapeutic effect of methanobactin, a potent bacterial copper-binding protein, in a rat model of Wilson disease, the Atp7b^-/- rat. Their results show a marked improvement of clinical, biochemical and ultrastructural abnormalities. They propose that methanobactin is a candidate drug for Wilson disease patients with severe liver disease.