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

Data are presented on several polymorphic genetic markers in 200 Greek gypsies. Polymorphic loci studied were: the ABO, MN, Rhesus, Kell and Duffy blood groups, hemoglobin, and ceruloplasmin. A survey for congenital malformations and hereditary diseases was also carried out on this group. The ABO, Rhesus, MN and Duffy system frequencies varied significantly from the figures obtained for the Greek population. However, there is a characteristic similarity between various gypsy groups studied in other nations and the distribution of polymorphic traits in the Punjab region of India. Cystic fibrosis, renal tubular acidosis, 21-hydroxylase deficiency, Hoty-Oram syndrome and homozygous beta-thalassemia were diagnosed within the gypsy group studied.
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PMID:Genetic structure of the Greek gypsies. 10 65

An objective approach for monitoring the treatment of acute pulmonary exacerbation in cystic fibrosis was evaluated. Eleven biochemical markers of inflammation (erythrocyte sedimentation rate, neutrophil count, C-reactive protein, alpha-1 antitrypsin, haptoglobin, ceruloplasmin, fibronectin, alpha-1 glycoprotein, alpha-2 macroglobulin, C3, granulocyte elastase and anti-Pseudomonas IgG) were measured in blood serum and plasma from 46 cystic fibrosis patients with chronic Pseudomonas aeruginosa colonization before and after treatment. The overall outcome in each patient was evaluated by means of a pondered sum of clinical, chest X-ray and lung function scores. Biochemical markers were related to the overall clinical improvement: haptoglobin, ceruloplasmin, fibronectin and alpha-1 glycoprotein showed a good sensitivity (64-70%), specificity (60-70%) and positive predictive value (86-89%). Granulocyte elastase showed a similar sensitivity (67%) and positive predictive value (85%) but a lower specificity (33%). The negative predictive value was generally poor (32-39%). Our data suggest that the combined measurement of some markers of inflammation and of conventional clinical parameters, may help in evaluating the efficacy of anti-infective treatment in cystic fibrosis.
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PMID:Modification of some markers of inflammation during treatment for acute respiratory exacerbation in cystic fibrosis. 151 Nov 95

Crossed immunoelectrophoresis (X-IEP) revealed several abnormalities in serum proteins from patients with adult respiratory distress syndrome (ARDS), tuberculosis (TB), and cystic fibrosis (CF). The two quite different kinds of pulmonary disease, one acute (ARDS) and the other chronic (TB and CF) exhibited serum changes specific for each disease and abnormalities associated with inflammation and pathogenesis, in general. In ARDS sera, most proteins were extremely low, presumably due to leakage into the lungs through damaged tissue, while the acute-phase proteins, orosomucoid, alpha 1-antitrypsin, alpha 1-antichymotrypsin, and haptoglobin, were markedly high when compared to the overall protein pattern. The extremely high alpha 1-antichymotrypsin values were not seen in corresponding TB and CF sera. Numerous TB patients had elevated alpha 1-antitrypsin, alpha 1-antichymotrypsin, and haptoglobin, but only the alpha 1-antitrypsin population mean was significantly different from normal. Gc-globulin, ceruloplasmin, and beta-lipoprotein were higher and alpha 1-lipoprotein and inter-alpha-trypsin inhibitor lower than normal. All other quantitative serum changes were not statistically significant. Surprisingly, all TB patients belonged to the Gc-1-1 genotype in contrast to the Gc-1-1, Gc-1-2, Gc-2-2 polymorphisms of the other populations. CF homozygote sera revealed statistically significant increases in the acute-phase proteins, alpha 1-antitrypsin, alpha 1-antichymotrypsin, and haptoglobin, while orosomucoid, transferrin, IgA, and IgG tended to be higher than normal. The tendency for higher levels of transferrin indicated possible iron deficiency in some patients. In contrast, prealbumin, alpha 1-lipoprotein, and inter-alpha-trypsin inhibitor were significantly depressed in CF patients. CF heterozygotes shared the decrease of alpha 1-lipoprotein with the patients while exhibiting small but significant depressions of alpha 2-macroglobulin and IgG. Though not statistically significant, lowered concentrations of alpha 1-antitrypsin were evident for the heterozygotes.
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PMID:Protein abnormalities in adult respiratory distress syndrome, tuberculosis, and cystic fibrosis sera. 243 15

The authors present a system for the appraisal of the nutritional and inflammatory condition in patients suffering from cystic fibrosis (CF) in the phase of apparent inactivity of pulmonary infection, using a system of indices based on the quantification of some plasmatic proteins. The plasmatic appraisals of 4 visceral proteins (albumin, thyroxine-binding prealbumin, retinol-binding protein and transferrin) and, as well, of 5 proteins of the acute phase (alpha-1-acid glycoprotein, alpha-1-antitrypsin, alpha-2-macroglobulin, haptoglobin and ceruloplasmin) were obtained in a control group of 16 healthy children and in another of 14 children affected by CF. With the proteic plasmatic appraisals of the control group, the knowledge of their biological value and after a statistical-mathematical analysis, the most sensitive, specific and independent proteins were determined for evaluating the nutritional and inflammatory condition, obtaining two simple formulas which were denominated Nutritional-Inflammatory Prognostic Indices (NIPI) A and B (NIPI A = alpha-1-acid glycoprotein + haptoglobin/albumin + prealbumin; NIPI B = haptoglobin/albumin). From the analysis of the results, it can be deduced that the children with CF are affected by an inflammatory process, very probably infectious.
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PMID:[Evaluation of the inflammatory and nutritional status in children with cystic fibrosis]. 267 74

Bronchial secretions from 191 children with various respiratory diseases and from 7 healthy controls were tested for concentration of ceruloplasmin, haptoglobin and transferrin. Highest protein levels were observed in children with bronchiectasis and cystic fibrosis. The protein levels were correlated with the intensity of the inflammatory process.
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PMID:[Ceruloplasmin, haptoglobin and transferrin levels in bronchial secretions of children with chronic respiratory tract diseases]. 653 57

Postprandial levels of copper, ceruloplasmin, iron, total iron binding capacity, cholesterol, vitamin A, carotene, folic acid, vitamin C, albumin, and total globulins in plasma, of 25-OH-vitamin D in serum, and of glutathione reductase activity, an index of riboflavin status, in erythrocytes were determined in a group of 18 juvenile cystic fibrosis patients receiving specialized outpatient care with attention to diet, vitamin supplementation, and pancreatic enzyme replacement. Bone mineralization was assessed by radiographic and photon beam technique. In the plasma of cystic fibrosis patients, levels were elevated for copper, ceruloplasmin, total globins, and total proteins and were depressed for iron, vitamin D, vitamin A, carotene, and albumin. Cortical thickness was diminished in the patients, but bone density was not. For patients with cystic fibrosis, a relation was established between forced vital capacity and certain biochemical indices in plasma. As forced vital capacity decreased, plasma levels increased for copper, total globulins and total proteins and decreased for albumin.
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PMID:Some biochemical indices of nutrition in treated cystic fibrosis patients. 722 98

Cystic fibrosis patients are at risk for nutrient deficiencies from malabsorption related to exocrine pancreatic insufficiency. This research examined the copper homeostasis of children with cystic fibrosis. Our objective was to measure cytochrome oxidase and copper-zinc superoxide dismutase activities in mononuclear cells, neutrophils, and erythrocytes of adolescents with cystic fibrosis, as well as plasma copper and ceruloplasmin. Thirteen adolescents with pancreatic insufficiency caused by cystic fibrosis were compared with 10 age- and sex-matched control subjects. Serum copper concentrations and ceruloplasmin measurements were not significantly different between the two groups. Cytochrome oxidase activity was significantly lower in the mononuclear cells and copper-zinc superoxide dismutase activity was significantly lower in the neutrophils and erythrocytes of the cystic fibrosis group. Other measures of trace element status such as hemoglobin concentration, serum ferritin, serum zinc, glutathione peroxidase activity, and manganese superoxide dismutase activity were not different between the two groups. Reductions in the activity of two copper-dependent enzymes suggest abnormal copper homeostasis in this population.
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PMID:Reduced copper enzyme activities in blood cells of children with cystic fibrosis. 766 Nov 26

One laboratory reports low activities for 2 blood copper enzymes in subjects with cystic fibrosis (CF), which suggests that moderate copper deficiency is common in this state. The present study attempted to confirm this proposition in 3 ways: repeat the measures for 1 of the 2 copper enzymes (superoxide dismutase) in a new group of CF patients (males and females, N = 38), add another copper enzyme measure (plasma diamine oxidase) that has high sensitivity to copper status, and test if copper enzyme activities in CF patients rise by copper supplementation. The last test was performed plus or minus zinc supplementation since poor zinc status may contribute to poor copper status. The results for the first 2 aims supported the idea of poor copper status, as low activities were found for CF subjects for 2 copper enzyme activities, erythrocyte superoxide dismutase and plasma diamine oxidase (although normal activities were obtained for another copper enzyme, plasma ceruloplasmin, both as U/mL plasma or U/mg ceruloplasmin immunoreactive protein). For the last aim, copper enzyme activities were not altered by copper supplementation (6 weeks, 3 mg copper/d as copper-glycinate), plus or minus concurrent zinc supplementation (30 mg zinc/d as zinc-glycinate). Therefore, CF may cause a tendency to moderate copper deficiency, which may be due to abnormal copper metabolism not easily corrected by increased copper and/or zinc intake.
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PMID:Copper enzyme activities in cystic fibrosis before and after copper supplementation plus or minus zinc. 1468 39

The aim of this preliminary study was to determine specific proteins, related to inflammation process and nutritional status as well as to total antioxidant capacity, in children suffering from cystic fibrosis (CF). The study was performed on 17 nonhospitalized children (12 boys and 5 girls) with CF aged 3 months to 10 years, who were assisted at the Nutrition Service from Pedro de Elizalde Hospital. Transferrin, transthyretin, ceruloplasmin (Cp), haptoglobin, C-reactive protein (CRP) and fibrinogen were measured by single radial immunodiffusion techniques. Total antioxidant capacity (TAC) was determined by a decolorization assay. Statistical analyses were performed by the Student's t test. Transferrin and transthyretin values were lower in CF patients in comparison with data obtained from healthy children (reference group, RG). The decreased transferrin concentration and the tendency towards low plasma transthyretin values suggested an abnormal nutritional status. However, higher Cp and haptoglobin levels were shown in patients than in RG. The fact that 23 and 50% of patients exceeded the desirable values for fibrinogen (<285.0 mg/dl) and CRP (<0.2 mg/dl), respectively, should be highlighted. The TAC (mM; Trolox equivalents) was shown to be lower in the CF group than in RG. The diminished TAC concomitant with an increased plasma Cp concentration would exacerbate the inflammatory status and could explain the depression of the immune system. These preliminary results could explain the need to include biochemical and functional parameters in the early nutritional status evaluation in CF patients in order to use appropriate nutritional and pharmacological therapies and consequently to improve their survival and quality of life.
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PMID:Inflammatory biomarker profile in children with cystic fibrosis: preliminary study. 2054 Aug 24