Storage copper

Reduction of N()2 to NO Reduction of N20 to N2 Copper storage Transcription factor Hydroxylation of Dopa Production of Protoporphyrin IX Hormone signalling Oxidation to methanol Production of signal peptides Copper pump Copper transfer... 

Miltimore, J.E., C.M. Kalnin, and J.B. Clapp. 1978. Copper storage in the livers of cattle supplemented with injected copper and with copper sulfate and chelated copper. Canad. Jour. Anim. Sci. 58 525-529. 

Metabolic disorders such as Wilson s disease (copper storage disease) and haemochro-matosis (iron overload disease),... 

Ceruloplasmin is involved in copper storage and transport as well as in iron mobilisation and oxidation. Among the blue oxidases it is unique since it contains, in addition to the usual motif of a type 1 combined with the trinuclear cluster, two other type 1 coppers. Electron transfer occurs, however, only between five of the six copper ions since one of the type 1 centres is not catalytically relevant due to its too high redox potential. The redox potentials of the centres were determined and possible electron transfer pathways among the copper sites were discussed.101... 

Copper Essential to all organisms constituent of redox enzymes and hemocyanin." Very toxic to most plants highly toxic to Invertebrates, moderately so to mammals. Pollution from industrial smoke and possibly from agricultural use. Wilson s disease, genetic recessive, results in toxic increase in copper storage. 

Copper proteins are involved in a variety of biological functions, including electron transport, copper storage and many oxidase activities. A variety of reviews on this topic are available (Sykes, 1985 Chapman, 1991). Several copper proteins are easily identified by their beautiful blue colour and have been labelled blue copper proteins. The blue copper proteins can be divided into two classes, the oxidases (laccase, ascorbate oxidase, ceruloplasmin) and the electron carriers (plastocyanin, stellacyanin, umecyanin, etc.). 

Wilson s disease is a copper storage disorder that is apparently due to an inherited lesion in the copper excretion mechanism. One in 200-400 persons is a carrier of the disease. Diagnosis may be made by measuring serum ceruloplasmin levels. Whereas normal serum ceruloplasmin is 200-400 mg/L, in Wilson s disease patients it is well below 200 mg/L. Liver copper in these patients (determined by biopsy) is more than 250 /xg/g, whereas normal individuals show a value of only 20-45 /xg/g. Liver function deterioration is the most prominent symptom of Wilson s disease. Treatment includes chelation therapy with penicillamine. 

The primary organs for copper storage are the liver and spleen, where the metal is found in the cytosol in superoxide dismutase see Copper Proteins with Type Sites) or metallothionein see Metallothiondns) In response to a copper challenge, yeast adaptively synthesizes metallothionein to detoxify the metal. Copper is also bound, transported, and assimilated into tissues by ceruloplasmin. 

Pollution from industrial smoke mid possibly from agricultural use. WSson s disease, genetic recessive, results in toxic increase in copper storage. 

Wilson s disease is a genetically determined, autosomal recessive copper storage disease with a reduced discharge of copper into the bile. Due to pathological copper deposits in the liver and brain as well as various other organs, sequelae develop above all in the liver and CNS. The other affected organs are generally involved in the disease as late manifestation. The chromosomal defect is still not fully clarified. 

Many of these copper proteins are enzymes, and the copper is a part of their active group (Nos. 1, 10-14 in Table 4), while others have no known enzyme activity (Nos. 2-9). As far as is known, none of these proteins functions as a respiratory carrier, as hemocyanin does in mollusks. It has been suggested, but not proven, that the human liver copper protein of Morell et al. (M32) and the hepatic mitochondrocuprein of Porter et al. (P13, P15) may function as copper storage proteins, similar perhaps to ferritin in the case of iron. 

A copper storage tank containing dilute H2S04 at pH = 0.1 is blanketed with H2 gas at 1 atm. Calculate the maximum Cu2+ contamination of the acid expressed as aCu2+. (Ref 11)... 

Copper thioneins have two functions, namely, copper storage and detoxification. ... 

Dick AT (1953) The control of copper storage in the liver of sheep by inorganic sulphate and molybdenum. Aust Vet J 29 233-240. 

The extent of the adverse effect of high sulfur intake in the diet of animals depends on species, the source of sulfur, and the concentration in feedstuffs. After depletion of the copper storages of the body, a sulfur exposure causes a secondary copper deficiency, especially in ruminants. In practice, it is difficult to distinguish between direct sulfur-induced toxic effects and secondary copper deficiency symptoms. 

Ceruloplasmin Binds copper appears to be more important as a copper storage pool than as a transport protein integrates iron and copper homeostasis... 

The effect of traces of copper on oat-seedlings, grown in a copper-deficient medium, is shown in Fig. 11.4. Plainly, too little copper is bad for growth, and so is too much. Until recognized as such, copper-deficiency was the cause of many a crop failure in the reclaimed areas of Holland and Denmark. Copper-deficiency in farm animals leads to anaemia, demyelination of the spinal cord, and loss of pigmentation. Excess of copper storage in the liver of sheep leads to haemolysis and death. Sheep, fed on a diet deficient in copper, lose the crimp in their wool. Because it is the crimp that makes fine wool saleable, this causes economic loss to farmers (see Fig. 11.2). 

Interaction of trace metals with one another or with other ions Mg as calcium antagonist, A1 binding phosphate in renal insufficiency, Zn interacting with copper (reduction of copper storage in Wilson s disease) and with cadmium intoxication, Se protecting against mercury intoxication, etc. [43,50,60,64]. 

High copper storage with intoxication is observed in animals fed a diet rich in copper sulfate with low molybdenum. In contrast, copper deficiency is observed in animals fed a normal-copper, high-molybdenum diet. The mechanism of interaction between the two metals is not clear because although copper administration to animals with high-molybdenum... 

Another disease related to a dysfunction in copper metabolism is Wilson s disease. Unlike in Menkes patients, copper in Wilson s patients is readily absorbed through the intestine and into the ceU. Within the cell, however, abnormally low levels of the copper-storage protein cemloplasmin are present. As a result, copper accumulates in the cytosol and is eventually released into the blood stream. The clinical manifestations of Wilson s disease are liver disease and neurological damage. 

A copper storage protein found in the brain See also copper... 

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