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)

There is a very important modification of the plasma protein equilibrium during an inflammatory reaction. Most of the proteins, except the immunoglobulins, have their biosynthesis or catabolic rate modified. The plasma concentration of these proteins may be increased, decreased or equal to the normal values. Clinically, the term "Acute Phase Protein" APP is reserved for the proteins whose plasma concentration is at least 50 per cent higher than normal values. These APP are: alpha-1 acid-glycoprotein, alpha-1 proteinase inhibitor, alpha-1 antichymotrypsin, haptoglobin, ceruloplasmin, fibrinogen, C-reactive protein, serum amyloid A protein. Interleukin-1 induces the APP's hepatic biosynthesis. The APP and, more generally, a lot of plasma proteins play a role during inflammatory reaction. They have some real functions of metabolic regulators. The functions result from the interaction of these proteins with ligands of various origins which give "protein-ligands" complexes. These complexes are cleared by the RES or by the hepatocyte. The results are protease inhibition, neutralization of toxic molecules such as hemoglobin or the superoxide anion, clearance of cell membranes and chromatin. The interaction of plasma proteins and particularly the APP's with different ligands issued from the inflammatory site, is an example of physiopathological self regulation.
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PMID:[Proteins of the inflammatory reaction. Regulatory functions]. 245 87

Wilson's disease is an autosomal recessive inherited disorder of hepatic copper metabolism resulting in liver disease and/or neuropsychiatric disease. The diagnosis of neurological disease is straightforward if the following symptoms are present: Kayser-Fleischer rings, typical neurological symptoms and low serum ceruloplasmin levels. The diagnosis is more complex in patients presenting with liver diseases. None of the commonly used parameters alone allows a diagnosis with certainty. A combination of various laboratory parameters is necessary to firmly establish the diagnosis. In the future, limited mutation analysis may play an important diagnostic role. Recently, a group of international experts has proposed a score based on a variety of tests and clinical symptoms. The validity of this score needs to be assessed prospectively. Treatment requires life-long administration of copper chelators (d-penicillamine, trientine). A frequently used alternative is zinc. None of these treatments has been tested by prospective randomized controlled studies. Liver transplantation is reserved for severe or treatment-resistant cases with advanced liver disease, whilst experience with refractory neuropsychiatric disease is limited.
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PMID:Review article: diagnosis and current therapy of Wilson's disease. 1472 7

The duodenal switch (DS) procedure is a type of restrictive-malabsorptive bariatric surgery that is typically reserved for severe morbidly obese people (body mass index >50 kg/m(2)) with obesity-related comorbidities, when diet, lifestyle changes, and pharmacologic therapy fail to achieve adequate weight loss. Patients who undergo the DS procedure are at risk for malabsorption, malnutrition, and nutrient deficiencies. Copper deficiency is a commonly reported long-term complication of Roux-en-Y gastric bypass (RYGB) surgery. However, data are limited on copper deficiency-associated complications and their treatment in DS patients. This article presents a case of a patient who developed hypocupremia with associated pancytopenia, myeloneuropathy, and leukoencephalopathy following DS and reviews the literature related to the pathophysiology of copper deficiency and copper replacement in bariatric surgery patients. When severe diarrhea was present, intravenous elemental copper 4 mg (as cupric chloride)/d in addition to daily oral copper gluconate was necessary to correct the hypocupremia and improve the hematologic indices and neurologic symptoms of copper deficiency. When diarrhea subsided, oral elemental copper 4 mg (as copper gluconate) 3 times daily maintained normal serum copper concentrations and avoided the relapse of severe neurologic dysfunction. Regular monitoring of serum copper and ceruloplasmin concentrations is recommended following DS surgery to detect any copper deficiency before irreversible neurologic damage occurs. Long-term copper supplementation is likely necessary to maintain normal copper status in DS patients.
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PMID:Neurologic dysfunction and pancytopenia secondary to acquired copper deficiency following duodenal switch: case report and review of the literature. 2194 41