Wilson Copper

The special interest is the determination of Fe, as the acceleration of sclerosed process is connected with the accumulation of Fe in this organ. Copper is accumulated in tissues at the genetic caused diseases (Wilson Disease, Hemochromatosis). This is the alteration in elemental ratios (e.g., Cu/Zn), that is the marker of pathological process. 

Alloys containing only a few per cent of zinc may fail if the stresses are high and the environment sufficiently corrosive. Most types of brass, besides the plain copper/zinc alloys, appear to be susceptible to stress corrosion. An extensive investigation of the effect of additions to 70/30 brass was carried out by Wilson, Edmunds, Anderson and Peirce , who found that about 1% Si was markedly beneficial. Other additions were beneficial under some circumstances and none of the 36 additions tested accelerated stress-corrosion cracking. Further results are given in later papers ... 

Werner s coordination theory, 1, 6 Whewellite structure, 6, 849 Wickmanite structure, 6, 849 Wilkinson s catalyst, 6, 239 Wilson s disease, 5, 721 copper, 6,648 removal, 6,769 copper complexes, 2,959 copper metabolism, 6,766 radiopharmaceutical agents, 6,968 Wolfram s red salt, 5,427 Wurzite... 

Wilson and Lyle [36] recently reported the synthesis and use of a new C2-symmetric 2,2 -bipyridyl ligand that answered these steric demands, since, associated to copper(I)salts, high diastereoselectivities and enantioselectivities trans/cis = 80/20 and 82% ee) could be achieved in the benchmark... 

Wilson disease (MIM 277900) Mutations in the gene encoding a copper-dependent ATPase... 

Ceruloplasmin Binds Copper, Low Levels of This Plasma Protein Are Associated With Wilson Disease... 

Wilson Disease Is Also Due to Mutations in a Gene Encoding a Copper-Binding P-Type ATPase... 

The cause of Wilson disease was also revealed in 1993, when it was reported that a variety of mutations in a gene encoding a copper-binding P-type ATPase were responsible. The gene is estimated to encode a protein of 1411 amino acids, which is highly homologous to the product of the gene affected in Menkes disease. In a manner not yet fully explained, a nonfunctional ATPase causes defective excretion of copper into the bile, a reduction of incorporation of copper into... 

Treatment for Wilson disease consists of a diet low in copper along with Ufelong administration of penicillamine, which chelates copper, is excreted in the urine, and thus depletes the body of the excess of this mineral. 

Ceruloplasmin contains substantial amounts of copper, but albumin appears to be more important with regard to its transport. Both Wilson disease and Menkes disease, which reflect abnormahties of copper metabohsm, have been found to be due to mutations in genes encoding copper-binding P-type ATPases. 

Certain oxides of divalent metals, those of ZnO, CuO, SnO, HgO, and PbO, form cements that are hydrolytically stable in addition MgO, CaO, BaO and SrO form cements that are softened when exposed to water. Compressive strengths of these materials range from 26 to 83 MPa, the strongest being the copper(II) and zinc polyacrylate cements (Table 5.1). Crisp, Prosser Wilson (1976) found that for divalent oxides the rate of reaction increased in the order... 

Brant, P. J. Wilson, A. D. (1985). Reaction cements as materials for the sustained release of trace elements into the digestive tract of cattle and sheep. I. Copper release. Journal of Veterinary Pharmacology Therapeutics, 8,... 

Copper appears as the a2-globulin ceruloplasmin in the human body (Sarkar 1994). Deficiency of this protein in serum is characteristic of both Menkes and Wilson s diseases. Wilson s disease is an abnormal storage of Cu(II) in body tissues. Cu(II) in biological material can be determined by spectrophotometry or by FAAS, ceruloplasmin in serum by a spectrophotometric method. 

Wilson s disease is another autosomal recessive disease leading to cirrhosis. Protein abnormalities result in excessive copper deposition in body tissues. The faulty protein is responsible for facilitating copper excretion in the bile, so copper accumulates in hepatic tissue. High copper levels within hepatocytes are toxic, and fibrosis and cirrhosis may develop in untreated patients. Those with Wilson s disease usually present with symptoms of liver or neurologic disease while still in their teens. 

Wilson s disease A disorder of copper metabolism characterized by cirrhosis of the liver and neurologic manifestations a potentially fatal genetic disorder that causes the body to retain copper. 

Using 1,4,8,11-tetraazacyclotetradecane, the structure of complex (800) (distorted trigonal planar Cu-Cu 6.739 A) was determined. Reactivity with 02 was investigated to demonstrate the formation of trans-l,2-peroxo species.585 As part of their work with copper(I) complexes with 02, the structure of a dicopper(I) complex ((801) distorted tetrahedral 7.04 A), supported by macrocyclic ligand environment, was reported by Comba and co-workers. Tolman and co-workers structurally characterized a three-coordinate copper(I)-phenoxide complex (802) (planar T-shaped) that models the reduced form of GO.587 The copper(I) analogue [Cu(L)][CF3-SO3]-0.43MeOI I (803) of a copper(II) complex (534) was also reported to demonstrate the role of ligand framework conformability in CV /Cu1 redox potentials.434 Wilson and co-workers... 

Copper is associated with two important life-threatening diseases in man, the pathologies of both being due to defective intracellular copper transport. Menke s disease is characterized by progressive cerebral degeneration, essentially due to insufficient copper absorption, and Wilson s disease is due to excessive copper accumulation in liver, accompanied by liver disease and haemolytic crises. 

Figure 12.4 Proposed path for the intracellular transfer of Cu(I) by Atxl. Copper destined for incorporation into the vascular multicopper oxidase Fet3 requires both Ctrl and Ccc2. Cytoplasmic Cu(I)-Atxl, but not apo-Atxl, associates with the amino-terminal domain of Ccc2 and Cu(I) is transferred to the latter. (Inset) A proposed mechanism for the exchange of Cu(I) involving two- and three-coordinate Cu-bridged intermediates. The human homologues of Atxl (Hahl), Ccc2 (Menkes and Wilson s proteins) and Fet3 (ceruloplasmin) are likely to employ similar mechanisms. Reprinted with permission from Pufahl et al., 1997. Copyright (1997) American Association for the Advancement of Science.

In mammals, as in yeast, several different metallothionein isoforms are known, each with a particular tissue distribution (Vasak and Hasler, 2000). Their synthesis is regulated at the level of transcription not only by copper (as well as the other divalent metal ions cadmium, mercury and zinc) but also by hormones, notably steroid hormones, that affect cellular differentiation. Intracellular copper accumulates in metallothionein in copper overload diseases, such as Wilson s disease, forming two distinct molecular forms one with 12 Cu(I) equivalents bound, in which all 20 thiolate ligands of the protein participate in metal binding the other with eight Cu(I)/ metallothionein a molecules, with between 12-14 cysteines involved in Cu(I) coordination (Pountney et ah, 1994). Although the role of specific metallothionein isoforms in zinc homeostasis and apoptosis is established, its primary function in copper metabolism remains enigmatic (Vasak and Hasler, 2000). 

Wilson s disease. Penicillamine is a chelating agent that removes excess copper in patients with Wilson s disease. From in vitro studies that indicate that one atom of copper combines with two molecules of penicillamine, it would appear that 1 g of... 

P-type copper transporters are important for neural function. Wilson s and Menke s diseases have major neurological components (Ch. 45). The Wilson s disease gene codes for a transporter, expressed chiefly in liver, that probably functions in Cu2+ excretion. The Menke s disease gene codes for a closely related transporter that regulates intestinal Cu2+ absorption [32],... 

Parkinson s disease (PD) is a hypokinetic movement disorder 766 Huntington s disease is a hyperkinetic movement disorder 771 Wilson s disease is a disease of copper accumulation 773 Dystonia is characterized by sustained muscle contractions 775 Many drugs and toxins induce movement disorders 776... 

Wilson s disease is an autosomal recessive disorder characterized by the accumulation of copper in liver and brain [21]. Hepatic involvement may result in liver cirrhosis and hepatic cancer. The deposition of copper in the basal ganglia results in a variety of movement disorders, including... 

FIGURE 46-7 Kayser-Fleischer ring in a young boy with Wilson s disease. The brown ring at the edge of the cornea is due to cornea] copper deposition. Courtesy of Dr Jorge Juncos (Emory University). 

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