title: A microbial peptide to rescue severe and fulminant Wilson disease?
abstract: 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.
authors.affiliation: University of Paris 7, 75013 Paris, France. Electronic address: serge.erlinger@gmail.com.
authors.forename: Serge
authors.initials: S
authors.lastname: Erlinger
mesh_terms: D000251:Adenosine Triphosphatases; D000818:Animals; D027682:Cation Transport Proteins; D003300:Copper; D000073840:Copper-Transporting ATPases; D006527:Hepatolenticular Degeneration; D006801:Humans; D007093:Imidazoles; D008772:Methylococcaceae; D009842:Oligopeptides; D051381:Rats
chemical_list: D027682:Cation Transport Proteins; D007093:Imidazoles; D009842:Oligopeptides; C491147:methanobactin; D003300:Copper; D000251:Adenosine Triphosphatases; D000073840:Copper-Transporting ATPases