The need for agents to lower body copper in Wilson's disease, a disease which results from copper toxicity has been the driving force for the development of the effective anticopper drugs penicillamine, trientine, zinc, and now tetrathiomolybdate (TM). Because of its rapid action, potency, and safety, TM is proving to be a very effective drug for initial treatment of acutely ill Wilson's diseas...

To cite: Menzies S, Kirby B. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/ bcr-2015-212482 DESCRIPTION A 42-year-old woman presented with a 5-year history of multiple enlarging skin lesions. Her medical history was significant for Wilson’s disease, for which she had taken penicillamine for approximately 20 years. She had multiple small erythaematous keratotic papul...

Long term D-penicillamine therapy, especially when used to treat Wilson's disease has been shown to cause elastosis perforans serpiginosa, pseudoxanthoma elasticum perforans and other degenerative dermatoses. We report a 23-year-old male patient who presented with multiple firm papules, nodules over the neck, axillae, front of elbows for five years. He was a known case of Wilson's disease on lo...

The title fluoridogermanate, {[Cd(2)(C(6)H(18)N(4))(3)][GeF(6)]}(n), was synthesized hydro-thermally. The crystal structure comprises undulated cationic [Cd(2)(TETA)(3)](4+) chains (TETA is triethyl-ene-tetra-mine) propagating parallel to [101]. The central Cd(II) atom is six-coordinated in a CdN(6) set by three TETA ligands. The isolated [GeF(6)](2-) units, serving as counter-anions, occupy th...

Nine new mononuclear and polynuclear Cu(II) complexes [Cu(H2L(2))Cl]Cl·3H2O (1), [Cu(H2L(3))Cl]Cl·H2O (2), [Cu(H2L(4))Cl]Cl·2.5H2O (3), [Cu3(μ(3)-L(1))2(H2O)3](ClO4)2·H2O (4), [Cu4(μ-HL(1))4](ClO4)8·CH3OH·5H2O (5), [Cu2(HL(3))2](ClO4)2·2H2O (6a), [Cu2(μ-HL(3))2](ClO4)2·H2O (6b), [Cu2(μ-HL(3))(L(3))Cu(teta)](ClO4)3·2H2O (7) and [Cu2(H2L(3))2(ox)](ClO4)2·2H2O·2MeOH (8) containing [5-(2-pyridine-2...

Strong promoters cloned into transcriptional fusion vectors can adversely affect plasmid copy number. In this study, we investigated the use of transcriptional repressors, lacI and tetR, to stabilize the copy number of plasmids containing the lacUV5 and tetA promoters, respectively. Repression of these promoters was found to prevent plasmid copy number variation. Transcriptional strength of the...

Wilson disease (WD) is an autosomal recessive disease of copper metabolism due to mutations in the ATP7B gene on chromosome 131. Patients may show a disabling movement disorder characterized by extrapyramidal signs with dystonia and choreoathetosis, associated with psychiatric signs and liver involvement, usually occurring during the first decades of life. Copper chelation by D-penicillamine re...