Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: 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)

In a series of 100 adult patients with definite rheumatoid arthritis of at most 3 years' duration and with no previous penicillamine, gold or systemic corticosteroid treatment, 50 patients were treated with D-penicillamine and 50 with gold for one yar. The dose of penicillamine was 600 mg daily. Sodium aurothiomalate was given 50 mg weekly up to a total of 13 mg/kg and thereafter 50 mg once a month. In both treatment groups a statistically significant decrease in the number of painful and/or swollen joints, an increase in haemoglobin and a decrease in ESR, serum ceruloplasmin-, alpha1-acid glycoprotein-, IgG-, IgM- and IgA levels was observed. All the changes in these clinical and laboratory tests were of the same degree in both treatment groups. In the penicillamine group 12 out of 20 seropositive patients became seronegative and in another 5 the Waaler-Rose titre dropped clearly. In the gold group, 7 out of 16 seropositive patients became seronegative, and the Waaler-Rose titre dropped in another 5. An equal increase in the number of eroded joints in hands and toes was seen in the penicillamine and the gold group. Penicillamine was discontinued because of side effects in 13 patients (26%), and gold treatment in 15 (30%). Proteinuria and/or haematuria were the most common causes of discontinuation in the penicillamine group.
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PMID:Comparison of penicillamine and gold treatment in early rheumatoid arthritis. 10 90

In a controlled therapeutic trial 17 patients with active rheumatoid arthritis (stage II and III) were divided in two randomized groups. One group of 9 patients was treated with 900 mg of D(-)penicillamine (Trolovol) plus 1500 mg of salicylate per day, the other group of 8 patients with 10 mg of prednisolone plus 1500 mg of salicylate daily. Before and after 1, 2, 3, 4 and 6 months of treatment IgA, IgG, IgM and caeruloplasmin were estimated with immunodiffusion technique. In both groups the IgA and IgG levels remained unchanged; unter D-penicillamine, on the contrary, a statistically significant and continuous fall of IgM and caeruloplasmin was observed; prednisolone treatment induced only a temporary fall of caeruloplasmin. In the DPA treated patients, a significant correlation of IgM with caeruloplasmin and of caeruloplasmin with ESR was found. In both groups there was no correlation between joint count and caeruloplasmin, joint count and IgM, caeruloplasmin and copper, copper and ESR; in the prednisolone group no correlation between caeruloplasmin, ESR and IgM was observed. The correlations suggest that the remarkable fall of IgM and caeruloplasmin under D(-)penicillamine treatment is caused at least partially by direct interference of DPA with these plasma proteins.
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PMID:[Ceruloplasmin and immunoglobulins under controlled D-penicillamine therapy in rheumatoid arthritis]. 126 22

The Long-Evans Cinnamon rat is a mutant strain that contracts hereditary hepatitis and, eventually, spontaneous hepatocellular carcinoma. Because we found a corresponding gross copper accumulation in the liver of the rats, we examined whether the development of hepatitis in our rat system could be prevented by administration of D-penicillamine. D-Penicillamine is a copper-chelating agent and one of the drugs effective for human Wilson's disease, in which abnormal copper metabolism is also observed. The results show that D-penicillamine treatment inhibited the elevation of serum transaminases, suppressed abnormal histological changes in the liver and completely prevented the onset of hepatitis in the Long-Evans Cinnamon rats. We further found that the copper concentration in the liver and serum copper and ceruloplasmin levels were decreased, whereas the urinary copper level was increased in the D-penicillamine-treated Long-Evans Cinnamon rats. These findings demonstrate that the pathogenesis of hereditary hepatitis in Long-Evans Cinnamon rats is due to abnormal copper accumulation in the liver.
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PMID:D-penicillamine prevents the development of hepatitis in Long-Evans Cinnamon rats with abnormal copper metabolism. 137 Jan 62

The pathophysiology, symptomatology, and treatment of Wilson's disease are reviewed, and new approaches to drug management are discussed. Wilson's disease is a rare, autosomal recessive disorder that occurs between the ages of 6 and 60 years. Disturbances in copper metabolism may result in the accumulation of excess copper in the liver, the basal ganglia of the brain (lenticular degeneration), the kidneys, the cornea (Kayser-Fleischer rings), and other tissues. The diagnosis of Wilson's disease is frequently overlooked; nonspecific symptoms and multisystem involvement may mimic other disease states, such as neurologic and psychiatric disorders, and hemolytic anemia. Screening tests for Wilson's disease include 24-hour urinary copper levels, serum ceruloplasmin and copper assays, radioactive uptake of 64Cu, and liver biopsy. Current methods of therapy include the use of a chelating agent--penicillamine or trientine--for initial rapid decoppering. Penicillamine therapy has been associated with many adverse reactions, including worsening of the neurologic symptoms of the patient. Zinc is a useful agent for maintenance therapy. Investigational studies exploring the use of ammonium tetrathiomolybdate for initial rapid decoppering have shown promising results. Unless it is recognized and treated, Wilson's disease can cause severe symptoms and, ultimately, death. Initial rapid decoppering with chelating agents, such as penicillamine and trientine, followed by lifelong maintenance therapy with zinc is the current method of treatment.
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PMID:Pathophysiology and treatment of Wilson's disease. 179 20

Autooxidation of bovine-brain homogenate proved to be a good model to determine the antioxidant capacity of sera. It was measured in parallel with the level of ceruloplasmin and apotransferrin in sera of 35 healthy children and 20 premature babies (gestational age 28-33 weeks). Antioxidant capacity was very low in premature babies with a nadir on the 4th postnatal day. Lipid-peroxidation of bovine-brain homogenate could be inhibited in vitro by the addition of exogen antioxidant. In this assay MTDQ-DS, Cavinton, DPA-HC1, vitamin E, A, C showed different antioxidant activity.
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PMID:Serum antioxidant activity in premature babies. 224

The mechanism by which D-Penicillamine is effective in the treatment of rheumatoid arthritis has been investigated. The results indicate that D-Penicillamine in synergism with copper or ceruloplasmin in vitro inhibits the proliferation of T-lymphocytes and the activity of helper T-cells in supporting the generation of antibody-forming cells. This effect is mediated by the production of H2O2. The significance of these findings for in vivo processes is discussed.
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PMID:Suggested mode of action of D-penicillamine as an immunosuppressive agent in rheumatoid arthritis. 297 7

The clinical features and investigations of 17 patients were analysed. Thirteen of them were Chinese and the rest Indians. Their ages at presentation ranged from 8 to 63 years (mean 18.35 years). Thirteen patients (76%) were symptomatic; 8 with predominantly hepatic manifestations and 5 with neurological features. Four were asymptomatic siblings. At diagnosis, however, 10(59%) had features of liver involvement singly, 3 (18%) had neurological involvement alone and 4 (27%) had mixed presentations. Family histories were available in 15 patients; 26.9% of siblings had Wilson's Disease. Serum ceruloplasmin was low in 82% of the patients. 24-hour urinary copper was measured in 16 patients and was raised in all of them. About half the patients (41%) had evidence of concomittant renal tubular dysfunction with hypouricaemia and aminoaciduria. Three patients (18%) had joint involvement at presentation. All 17 patients were treated with Penicillamine. Complications due to therapy included pemphigus in one and toxic epidermal necrolysis and later a lupus like syndrome in another. The features of clinical improvement included fading of K-F rings, improvement of neurological signs and the normalisation of serum transaminases. One patient developed primary hepatocellular carcinoma 5 years after presentation. Delay in diagnosis was encountered in half of the patients reviewed. Being a treatable condition, Wilson's Disease, although rare, should always be thought of in patients with haemolysis, liver diseases or extrapyramidal disorders.
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PMID:Wilson's disease revisited in the tropics. 375 94

To test the hypothesis that ferroxidase I (ceruloplasmin) activity is essential for iron mobilization, adult rats were fed a copper sufficient diet with or without the chelating drugs D-penicillamine and triethylenetetramine for 120 days. By day 6 of treatment and for the remainder of the experiment the drug-fed rats showed low plasma copper concentration and low ferroxidase I activity. Plasma ferroxidase II activity in the DPA and TETA groups tended to be slightly lower than that of controls. No animals became anemic. Therefore, persistent low plasma ferroxidase I does not necessarily cause anemia in the adult rat.
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PMID:The effect of copper chelating drugs on liver iron mobilization in the adult rat. 686 Mar 30

Wilson's disease (WD), an inborn error of copper (Cu) metabolism, is now one of the leading liver diseases in children in India. The clinical presentation can be extremely varied viz.,--all forms of acute and chronic liver disease, minimal to severe neurological disease, psychiatric problems, bony deformities, hemolytic anemia and endocrine manifestations. A high index of suspicion is necessary along with a judicious battery of investigations for diagnosis. Hepatic copper estimation is the most reliable test but is not easily available in India. Liver biopsy may not be possible because of bleeding problems and histological features are often not diagnostic of WD. In the absence of hepatic Cu, a low ceruloplasmin, high 24 hour urinary copper and presence of KF rings aid in making the diagnosis. The mainstay of initial therapy is Cu-chelators like D-Penicillamine, and Trientine for reduction in body copper to sub-toxic levels. Subsequent maintenance therapy is necessarily lifelong with D-Penicillamine, Trientine or Zinc. Children on therapy must be monitored regularly for response, side-effects, compliance and rehabilitation. Response to therapy may be unpredictable, but acute and early presentations like fulminant hepatic failures have a poor outcome. All siblings must be screened for WD as early diagnosis and treatment result in a good outcome. The identification of the WD gene on chromosome 13 has led to the possible use of molecular genetics (haplotype and mutational analyses) in the diagnosis of WD. Parent groups/associations must take active part in holistic management of WD.
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PMID:Wilson's disease. 1242 Sep 12

A 17-year-old girl with haemolytic anaemia, parenchymal livel disease and gallbladder calculi, is reported. Kayser-Fleischer rings, transaminasaemia, deficiency of ceruloplasmin, increased cupriuria, and nodular cirrhosis of the liver, confirmed the diagnosis of Wilson's disease. Penicillamine therapy had to be interrupted a short time after it was started, because of penicillamine-induces acute psychotic episode. Zinc-sulphate has controlled Wilson's disease in the patient.
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PMID:[Wilson's disease]. 1797 25


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