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)

Twin males aged 24 years showed dementia, dysarthria, gait disturbances and involuntary movements, with slightly low levels of serum copper and ceruloplasmin, and markedly low excretion of urinary copper. We propose that the unique combination of dementia, dysarthria, gait disturbances, involuntary movements and abnormalities of copper metabolism does not fit any known nosological entity and constitutes a "new" syndrome different from Wilson's and Menkes' diseases.
J Neurol 1988 Sep
PMID:An unusual neurological disorder with abnormal copper metabolism. 306 63

Coagulation factor V is a high molecular weight plasma glycoprotein that participates as a cofactor in the conversion of prothrombin to thrombin by factor Xa. A phage lambda gt11 Hep G2 cell cDNA expression library was screened by using an affinity-purified antibody to human factor V, and 11 positive clones were isolated and plaque-purified. The clone containing the largest cDNA insert contained 2970 nucleotides and coded for 938 amino acids, a stop codon, and 155 nucleotides of 3' noncoding sequence including a poly(A) tail. The coding region includes 651 amino acids from the carboxyl terminus that constitute the light chain of human factor Va and 287 amino acids that are part of the connecting region of the protein. The predicted amino acid sequence agreed completely with 147 amino acid residues that were identified by Edman degradation of cyanogen bromide peptides isolated from the light chain. During the activation of factor V, several peptide bonds are cleaved by thrombin, giving rise to a heavy chain, a connecting fragment(s), and a light chain. The light chain is generated by the cleavage of an Arg-Ser peptide bond. The amino acid sequence of the light chain is homologous (40%) with the carboxyl-terminal fragment (Mr, 73,000) of human factor VIII. Both fragments have a similar domain structure that includes a single ceruloplasmin-related domain followed by two C domains. The carboxyl terminus of the connecting region, however, shows no significant amino acid sequence homology with factor VIII. It is very acidic and contains a number of potential N-linked glycosylation sites. It also contains about 20 tandem repeats of nine amino acids.
Proc Natl Acad Sci U S A 1986 Sep
PMID:Cloning of a cDNA coding for human factor V, a blood coagulation factor homologous to factor VIII and ceruloplasmin. 309 20

The acute response to injury and infection is manifested by increased synthesis of acute-phase proteins by the liver, an increased white blood cell count, fever, a negative nitrogen balance, and altered serum mineral levels (zinc, iron, and copper). This response is thought to be partially mediated by cytokines such as interleukin-1, but has not been well studied in head-injured patients. In this study, 25 patients were studied for evidence of the acute-phase response extending from hospital admission up to 21 days postinjury. The patients were divided into two groups to determine if severity of injury influenced the response. Group 1 consisted of nine patients with admission peak 24-hour Glasgow Coma Scale (GCS) scores of 4 or less; Group 2 consisted of 16 patients with admission peak 24-hour GCS scores of 8 or greater. All patients demonstrated some evidence of the acute-phase response. Serum alpha-1 acid glycoprotein, ceruloplasmin, and C-reactive protein levels were elevated on admission and throughout the study. Serum albumin and zinc levels were depressed on admission; zinc levels gradually normalized by Day 21 in both groups, but hypoalbuminemia was observed throughout the study period. Serum copper levels were normal on admission but increased to above normal in both groups by Day 11 postinjury. Urinary urea nitrogen excretion was elevated in both groups and peaked on Day 7 for Group 1 and Day 11 for Group 2 patients. The patients with admission GCS scores equal to or less than 4 had overall higher temperatures than were seen in those with GCS scores greater than or equal to 8 (p = 0.009). All patients but one had an elevated white blood cell count on admission. It is concluded that brain-injured patients with admission GCS scores of 3 to 4 and 8 to 14 demonstrate an acute-phase response which lasts for at least 3 weeks postinjury. It is speculated that this response is at least partially mediated by increased intraventricular interleukin-1 activity.
J Neurosurg 1988 Sep
PMID:The acute-phase response of the brain-injured patient. 313 34

Solutions of wheat germ agglutinin exclusively but incompletely react with serum glycoproteins containing N-acetylneuraminic acid, viz. alpha 2-macroglobulin, haptoglobin, haemopexin, immunoglobulin A, alpha 1-acid glycoprotein, ceruloplasmin, immunoglobulin M (in decreasing order) and others. The precipitation does not proceed stoichiometrically and depends on lectin and polyethyleneglycol concentration, temperature, pH-value, ionic strength, and matrix effects. Presumedly, the reaction is initiated by specific and electrostatic interactions of wheat germ agglutinin with sialic acid residues of the glycoprotein and followed by binding of N-acetylglucosamine residues. A minimal precipitation of albumin is due to its complex formation with glycoproteins via disulphide bonds. Although wheat germ lectin precipitation sensitively detects serum sialoproteins, its intensity does not reflect the amount of N-acetylneuraminic acid in serum glycoproteins, thus calling in question the analytical use of this lectin for the measurement of sialoglycoconjugates.
J Clin Chem Clin Biochem 1988 Sep
PMID:[Studies on the reaction of wheat germ agglutinin with serum glycoproteins. Lectins as reagents. III]. 319 77

Severely head-injured patients are hypermetabolic/hypercatabolic and exhibit many aspects of the postinjury acute-phase response. These patients have hypoalbuminemia, hypozincemia, hypoferremia, hypercupria, fever, and increased synthesis of acute-phase proteins such as ceruloplasmin and higher C-reactive protein levels. It has been suggested that increased interleukin-1 (IL-1) in the ventricular fluid may be responsible, at least in part, for these metabolic abnormalities. In the present study, serum albumin levels were evaluated throughout an 18-day study period in 62 head-injured patients receiving aggressive nutritional support. Hypoalbuminemia (mean +/- standard error of the mean 3.10 +/- 0.2 gm/dl; normal value 3.5 to 5 gm/dl) was observed upon hospital admission; these albumin levels continued to decrease until 2 weeks postinjury, despite aggressive nutritional support. This hypoalbuminemia may be mediated via altered endothelial permeability properties due to endothelial cell dysfunction caused by cytokines such as IL-1. Transendothelial movement of albumin was assayed using a pulmonary artery endothelial cell culture system. Both a crude macrophage supernatant derived from a murine P388D cell line having IL-1 activity (mIL-1) and human recombinant IL-1 (rIL-1) were tested. The amount of albumin transferred was time- and concentration-dependent, with maximal transfer at 24 hours and 20 U of mIL-1 per 0.5 ml of culture medium. Endothelial permeability changes observed after incubation with mIL-1 were confirmed using rIL-1. Compared to control cultures, 20 U of rIL-1 and 20 U of mIL-1 increased albumin transfer across endothelial monolayers 205% and 459%, respectively. These findings suggest that the mechanism of hypoalbuminemia seen after severe head trauma can be explained in part by IL-1-induced endothelial cell injury, resulting in enhanced endothelial permeability to albumin.
J Neurosurg 1988 Sep
PMID:Mechanisms and implications of hypoalbuminemia in head-injured patients. 326 27

The effects of aqueous and vitreous humors and plasma on the rate of auto-oxidation of a rabbit brain homogenate were measured. Both aqueous and vitreous humors from normal eyes increased, while plasma decreased the rate of oxidation in the homogenate. During endotoxin-induced ocular inflammation the copper (Cu) and iron (Fe) concentrations of both the aqueous and vitreous humors increased, most likely due to the influx of their plasma binding proteins, ceruloplasmin (Cu) and transferrin (Fe). As both proteins are known to be antioxidants, it was not surprising to find that the aqueous and vitreous humor from the inflamed eyes had significant antioxidant activity. This antioxidant activity correlated well with the concentrations of Cu and Fe in aqueous humor and Cu but not Fe in the vitreous humor throughout the time course of the inflammatory response. Thus, entry of plasma proteins through disrupted blood ocular barriers may function in protecting ocular tissues against the increased oxidation which occurs during inflammation.
Curr Eye Res 1986 Sep
PMID:Antioxidant activity of aqueous and vitreous humor from the inflamed rabbit eye. 349 Mar 55

The pharmacokinetics and pharmacodynamic effects of two doses of norethisterone (5mg and 3mg) used as a 'visiting pill' were investigated. There were no significant differences in the pharmacokinetics of the two doses except for the peak concentration achieved and the bioavailability as assessed by the area under the serum norethisterone concentration - time curve. Both doses were rapidly absorbed. Pharmacodynamic effects were minor. No change occurred in serum concentrations of total cholesterol, total triglycerides or HDL-cholesterol. The area under the serum glucose concentration--time curve and particularly the area under the serum insulin concentration--time curve were significantly increased as a result of treatment but no change occurred in the serum levels of glycosylated haemoglobin. SHBG concentrations in serum decreased on treatment whereas those of ceruloplasmin increased.
Contraception 1986 Sep
PMID:A pharmacokinetic and pharmacodynamic study of a 'visiting pill' containing norethisterone. 353 10

Ceruloplasmin, a copper-containing acute phase plasma protein, has been shown to be regulated by 13-cis retinoic acid in rats. Ceruloplasmin activity was significantly increased within 24 h and remained elevated for at least 72 h after a single injection of 13-cis retinoic acid. With daily injections of retinoic acid, the ceruloplasmin activity continued to increase for at least 4 d. After 4 d, the activity was four times control levels. In copper-deficient rats, the ceruloplasmin activity did not increase in response to retinoic acid unless copper was also given to these rats 8 h after retinoic acid. Actinomycin D blocked the retinoic acid-induced stimulation of ceruloplasmin activity in copper-sufficient rats, but in copper-deficient rats only about half of the increase was blocked when the rats were given copper or copper and retinoic acid. By use of pulse-labeling techniques, ceruloplasmin synthesis was shown to increase 1.5-fold after retinoic acid and this increase was blocked by actinomycin D. When vitamin A-deficient rats were repleted with 13-cis retinoic acid for 3 or 5 d, both the ceruloplasmin activity and synthesis were significantly stimulated when compared to the nonrepleted, deficient rats. Therefore, the dietary components, copper and vitamin A, play an important role in the regulation of plasma ceruloplasmin levels.
J Nutr 1987 Sep
PMID:Induction of ceruloplasmin synthesis by retinoic acid in rats: influence of dietary copper and vitamin A status. 365 40

Antioxidant system ceruloplasmin-transferrin (Cp-Tr) and malonic dialdehyde (MDA) concentration changes were studied in the rat serum after the exposure to hyperbaric oxygenation (HBO) at 4 atm for 25 min. 5 sessions of HBO led to an increase in serum MDA concentration. 5 HBO sessions were followed by the activation of Cp-Tr system. Afterwards MDA concentration began to decrease and by the 9th session even reached the initial levels. It is suggested that antioxidant system Cp-Tr takes part in the protection of the organism from toxic oxygen action.
Biull Eksp Biol Med 1987 Sep
PMID:[The ceruloplasmin-transferrin antioxidant system during hyperbaric oxygenation in rats]. 366 7

The administration of very low doses of bacterial endotoxin protects rats during exposure to hyperoxia and is associated with the induction of lung antioxidant enzyme activities. Copper-deficient rats have increased susceptibility to O2 toxicity, which may be related to their decreased lung superoxide dismutase activity (SOD) or decreased plasma ceruloplasmin concentrations. To determine whether endotoxin can protect against hyperoxia in this susceptible model, we exposed copper-deficient and control rats to a fractional inspiratory concentration of O2 greater than 0.95 for 96 h after pretreatment with 500 micrograms/kg of bacterial endotoxin or phosphate-buffered saline (PBS). Mortality in the copper-deficient and control rats given PBS and exposed to O2 for 96 h was 100%. Copper-deficient rats died significantly earlier during the exposure than controls. No mortality occurred in either group treated with endotoxin and hyperoxia despite the decreased activity of copper-dependent enzymes in the copper-deficient rats. Copper-deficient rats treated with endotoxin and exposed to hyperoxia did increase lung Cu-Zn-SOD activity, but activity remained below levels found in air-exposed controls. Mn-SOD activity was found to be induced above air-exposed controls in the copper-deficient rats treated with endotoxin and exposed to hyperoxia. Hyperoxic exposure resulted in a marked increase in plasma ceruloplasmin concentrations in the control rats, but no increases in ceruloplasmin occurred in the copper-deficient animals. Endotoxin protects copper-deficient rats from hyperoxia despite their decreased lung Cu-Zn-SOD activity, and decreased plasma ceruloplasmin.
J Appl Physiol (1985) 1986 Sep
PMID:Effects of bacterial endotoxin on protecting copper-deficient rats from hyperoxia. 375 84


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