Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:1.16.3.1 (ceruloplasmin)
 5,074 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Wilson disease presenting as fulminant hepatic failure, severe hemolysis and renal failure is rare in the literature. A ten-year-old boy--complaining of abdominal pain, jaundice, tea-colored urine, and anemia was admitted to this hospital; examination showed Kayser-Fleischer rings, anemia associated with hemolysis, mildly elevated serum transaminases, extremely elevated bilirubin levels, low serum ceruloplasmin level, slightly elevated serum copper, excessive 24-hour urine copper excretion, and severe renal function insufficiencies. Under the impression of Wilson disease with fulminant hepatic failure, the patient was treated by oral D-penicillamine 1 gm per day, intravenous zinc sulphate (about 8 mg per day elemental zinc), and given other supportive treatment. Unfortunately, the patient died of hepatic failure complicated with septic shock 21 days after the onset of symptoms. Autopsy found liver copper content was 586.92 ug/gm dry weight and kidney copper content: 300.19 ug/gm dry weight, abnormally high as compared with normal tissue. A review of the literature led to conclusion that the best treatment for Wilson fulminant hepatic failure is liver transplantation.
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PMID:[Wilson disease presenting as fulminant hepatic failure, acute hemolytic anemia and renal failure: report of one case]. 226 86

The concentrations of three trace elements (iron, copper and zinc) involved in hematopoiesis were studied in 200 patients with iron-deficit anemia, hemolytic anemia and Biermer's anemia, in comparison with a group of normal subjects. The method used was atomic absorption spectrophotometry. The results obtained showed that in iron-deficit anemia resistant to iron therapy the copper and ceruloplasmin serum concentrations are at the lowest limit of normal. This copper deficit has a negative effect on the disease by preventing hemoglobin synthesis. In iron-deficit anemias responding to iron therapy the relatively low (about 90 gamma %) zinc values have a negative effect by blocking the iron in the iron reserve. In hemolytic anemia the very high zinc serum concentrations (over 200 gamma %) may lead to a shortening of the erythrocytes life span due to its "entatic state". In Biermer's anemia zinc by its high concentration has a positive role owing to carbonic anhydrase which eliminates more rapidly carbon dioxide from the organism.
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PMID:Possible correlation between the zinc and copper concentrations involved in the pathogenesis of various forms of anemia. 228 64

The purpose of this study was to determine what levels of starch or glucose replacement for fructose in the copper-deficient diet (copper) can minimize the fructose-copper interaction. Experimental diets contained either 100% fructose as the carbohydrate source, or the fructose was partially replaced with 50% starch, 50% glucose, 75% starch, or 75% glucose. Diets were either copper adequate (7-8 ppm) or inadequate (less than 1 ppm). Male weanling rats were fed their respective diet for 5 weeks and then fasted overnight. After decapitation, blood was collected and liver and heart were removed. Plasma copper was significantly reduced and ceruloplasmin was not detected in all copper-deficient groups. Copper deficiency increased plasma cholesterol, as well as heart and liver weight in the glucose groups, but not in the starch groups. Those organ weights were heavier in glucose-copper than starch-copper rats. Erythrocyte copper-zinc-superoxide dismutase activity was greater in starch-copper rats. Erythrocyte copper-zinc-superoxide dismutase activity was greater in starch-copper than glucose-copper rats regardless of carbohydrate amount. Hepatic copper concentration of the group fed starch-copper was twice levels observed in glucose-copper. The 50% glucose rats had lower hepatic copper than the 75% glucose rats. Hepatic copper-zinc-superoxide dismutase activity showed patterns similar to hepatic copper. Cardiac copper was greater in starch-copper than glucose-copper rats. Cardiac copper-zinc-superoxide dismutase activity was equally reduced in all copper-deficient groups. The 50% starch-replaced diet was more effective in minimizing copper deficiency than the 75% glucose-replaced diet. This poorer improvement of copper deficiency by glucose than starch may partially be due to a more severe reduction of food intake in glucose than in starch diets.
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PMID:Comparison of copper status in rats when dietary fructose is replaced by either cornstarch or glucose. 234 47

Anticonvulsant therapy is known to be associated with elevated plasma copper and ceruloplasmin levels. To investigate the effect of long-term phenytoin and/or phenobarbital administration, plasma copper, zinc, and ceruloplasmin levels were measured in two groups of patients with developmental disorders who were either living at home (study 1) or institutionalized (study 2). Plasma copper and zinc levels were measured using an atomic absorption spectrophotometric method, and ceruloplasmin levels were determined using a method measuring oxidase activity (study 1) and an immunodiffusion plate method (study 2). In both studies, the mean plasma copper and ceruloplasmin levels of the anticonvulsant groups were significantly higher than those of control groups, while the mean plasma zinc levels for the anticonvulsant and control groups were similar. Possible change in the tissue zinc/copper ratio among the patients receiving long-term anticonvulsants is discussed.
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PMID:Effect of long-term administration of anticonvulsants on copper, zinc, and ceruloplasmin levels. 242 May 49

Twenty-two children with Down syndrome (DS), 8 boys and 14 girls, in the age range 5 to 15 years were investigated and compared with a control group of 22 healthy children, 9 boys and 13 girls of the same age group, 9 of them being siblings of patients with DS. Concentrations of iron, copper and zinc in serum were determined by atomic absorption spectrophotometry and serum proteins were quantitated by the radial immunodiffusion technique. The subjects with DS had significantly lower mean serum iron (P less than 0.01) and zinc (P less than 0.001) than the healthy controls. Four DS children had serum iron values that fell below the normal range. In more than 60 per cent of the DS patients the zinc concentration fell below the normal range. The children with DS had significantly higher mean serum copper (P less than 0.05) but lower serum iron (P less than 0.05) and zinc (P less than 0.05) levels than their healthy siblings living in the same family at the time of examination. The DS patients as a group had higher levels of caeruloplasmin (P less than 0.01), haptoglobin (P less than 0.001), orosomucoid (P less than 0.001) and alpha 2-macroglobulin (P less than 0.001) than the healthy controls and compared with their siblings. Except for prealbumin and retinol-binding protein (RBP), no age-related variation in the serum concentrations of the studied proteins was found in the DS patients. Albumin, prealbumin, RBP and transferrin levels were similar in the two study groups.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Trace elements and transport proteins in serum of children with Down syndrome and of healthy siblings living in the same environment. 244 24

Interleukin 1 (IL 1) production is stimulated by infection, cellular injury, and inflammation. This cytokine directs a wide spectrum of host responses. Human interleukin 1 alpha (IL 1 alpha) was used to examine the time course of effects on zinc metabolism as part of the acute phase response. IL 1 produced a transient depression in the serum zinc concentration and increased serum ceruloplasmin. Metallothionein levels were increased in liver 14-fold after IL 1. Increased expression of metallothionein-1 and -2 genes following IL 1 were observed in liver, bone marrow, and thymus. Pulse-labeling experiments with i.v.-administered 65Zn showed that IL 1 drastically altered zinc distribution kinetics among tissues. More 65Zn was taken up (and/or retained) by the liver, bone marrow, and thymus 6 h after IL 1, whereas correspondingly less 65Zn was found in bone, skin, and intestine. Uptake by other tissues was not affected by IL 1. Chromatography of cytosol from tissues with increased 65Zn uptake suggests the IL 1-induced redistribution may be driven by enhanced metallothionein synthesis. Collectively, the results show that IL 1 regulates zinc metabolism and may direct its preferential, tissue-specific distribution via elevated metallothionein-1 and -2 gene expression.
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PMID:Tissue-specific regulation of zinc metabolism and metallothionein genes by interleukin 1. 245 83

Two-wk-old broiler chicks were inoculated via crop intubation with Eimeria acervulina at two doses: 10(5) or 10(6) sporulated oocysts/bird or with Eimeria tenella at a dose of 10(5) sporulated oocysts/bird. Serum and liver samples were collected on days 3 and 6 post-inoculation (PI). There were no significant changes in serum or liver zinc, copper, and iron concentrations in any of the infected groups by 3 d PI. However, on d 6, PI serum protein was significantly reduced in all of the infected groups compared to their pair-fed controls. The chicks infected with E. tenella had significantly reduced serum zinc (1.20 vs 1.77 micrograms/mL) and iron (0.44 vs 1.28 micrograms/mL) concentrations and significantly elevated serum copper (0.28 vs 0.17 micrograms/mL) and ceruloplasmin levels (20.33 vs 11.11 micrograms/mL) compared to their pair-fed counterparts. Those chicks infected with E. acervulina (10(6) oocysts/bird) exhibited significantly reduced serum iron concentration by 6 days PI (0.90 vs 1.14 micrograms/mL). Liver zinc was significantly increased in the chicks infected with E. tenella (349 vs 113 micrograms/g dry liver wt), as was copper (24 vs 19 micrograms/g), whereas liver iron concentration was significantly reduced (172 vs 243 micrograms/g) compared to pair-fed controls. At both dose levels, the chicks infected with E. acervulina exhibited a significant reduction in liver iron by 6 d PI. Hepatic cytosol metals generally reflected whole tissue levels. Metallothionein (MT)-bound zinc was significantly elevated in the chicks infected with E. tenella. Iron bound to a high molecular weight, heat-stable protein fraction (presumably cytoplasmic ferritin) was significantly reduced in chicks infected with E. acervulina, as well as those infected with E. tenella. Collectively, the changes in serum zinc, copper, and iron concentrations, as well as the changes in hepatic zinc and MT-zinc concentrations in the chicks infected with E. tenella were similar to changes evoked during an acute phase response to infection. It is possible that a secondary bacterial infection or inflammation stemming from erosion of the lining of the cecum may play a role in the response of trace element metabolism to the E. tenella infection.
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PMID:Serum and liver zinc, copper, and iron in chicks infected with Eimeria acervulina or Eimeria tenella. 248 59

A deficiency or an excess of some elements in the diet is reported to modify the concentration of cholesterol in plasma, and, conversely, a reduction of cholesterol in the diet decreases zinc in plasma. We have studied the distribution of elements Na, K, Ca, Mg, Fe, Cu, Zn, S, P, and Mn in the tissues, plasma, heart, aorta, lung, liver, spleen, kidney, thymus, and brain of New Zealand White rabbits (NZW) and of Watanabe Heritable Hyperlipidemic rabbits (WHHL). The WHHL rabbits had a massive hypercholesterolemia (7.45 +/- 1.2 g/L) induced by a lack of liver low density lipoprotein receptors. The concentrations of elements in the tissues of the control NZW rabbits were very similar to those found in the normal rat. In WHHL, compared to NZW, besides the very important increase of total phosphorus in plasma explained by the augmentation of phospholipids, there was an increase of plasma copper (+44%) and zinc (+36%). The other noticeable changes were an increase of iron in heart (+19%), sulfur, and zinc in liver (+15% and +18%). The other changes observed in WHHL rabbits were, besides the increase of ceruloplasmin, the increase of vit E (+468%) and MDA (+62%). In conclusion, despite a massive increase of lipids in plasma, there was no major disturbance of element distribution in WHHL rabbits.
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PMID:The distribution of elements in the tissues of Watanabe Heritable Hyperlipidemic Rabbits. 248 83

The influence of lead exposure, iron deficiency, or their combination on certain biochemical parameters in blood, plasma, and urine of rats was investigated in an attempt to identify the specific diagnostic tests of the two conditions and to draw a possible interrelationship between the two factors. The decrease in blood-glutathione peroxidase activity, -packed cell volume, plasma-ceruloplasmin, and-Fe levels and increase in urinary excretion of delta-aminolevulinic acid, plasma-cholesterol, and-total Fe binding capacity occur under Fe deficiency as well as Pb intoxication. However, increase in the activity of blood delta-aminolevulinic acid dehydratase (ALAD) without any change in blood zinc protoporphyrin (ZPP) level appears to be a specific effect of Fe deficiency that could be distinguished from Pb intoxication, a condition characterized by the inhibition in blood ALAD activity accompanied by an increase in blood ZPP level. The linear regression analysis of the data showed that the blood Pb and plasma free cholesterol levels increase with the decrease in plasma Fe level.
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PMID:Interrelationship between iron deficiency and lead intoxication (Part 1). 248 14

The status and transfer of metals across the rat placenta were studied by subcellular and molecular fractionations of this organ at 2 and 24 h after iv injection of radiolabeled metals. The soluble and nuclear fractions showed higher contents of copper and zinc, whereas most of the nickel was associated with the soluble fraction. Cadmium was almost evenly distributed between the microsomal and nuclear fractions. Gel filtration of the soluble fractions showed nickel associated with an unknown low molecular weight form; zinc with high molecular weight proteins; copper with metallothionein, ceruloplasmin, and high molecular weight proteins; and cadmium with high molecular weight proteins and metallothionein.
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PMID:The metabolism of metals in rat placenta. 248 64


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