Ceruloplasmin (CER) is a glycoprotein composed of a single polypeptide chain with an MW of ~132 kDa. CER is a copper- oxidizing enzyme, synthesized in the liver; it binds about 95% of circulating copper and transports it to tissues. CER acts on iron (Fe) regulation and other metal ion status by exerting an antioxidant action as it inhibits metal ion catalyzed oxidation on membrane lipids. The reference values in plasma for adults, according to the IFCC international standardization (CRM 470), are 0.2–6.0 g/L. Clinically significant decreases in plasma concentration of CER are present in hereditary synthesis defect, which is very rare, or in secondary deficits, such as those found in Wilson’s disease (an autosomal recessive defect of copper metabolism). Since CER is an acute-phase protein, physiological concentrations of CER can be observed in conjunction with inflammatory states. In addition, the synthesis of the protein is estrogen- dependent and, therefore, high concentrations of CER are found in pregnancy and during estrogen administration in menopause, making the diagnostic specificity of this test rather low and not allowing to use the measurement of the protein as a screening test for Wilson’s disease. CER values <0.05 g/L are strongly indicative for the diagnosis of disease; however, it should be noted that concentrations within the reference range do not allow to exclude the presence of Wilson’s disease for the reasons stated above. The diagnostic value of low plasma concentrations of CER increases significantly when associated with the presence of Kaiser–Fleischer corneal rings. In the absence of this sign, as commonly occurs in the hepatic manifestation of the disease, on the one hand, low concentrations of CER cannot be considered diagnostic because they could be due to other pathologies (auto immune hepatitis, celiac disease), on the other hand, being CER an acute phase positive protein, during inflammation its concentrations could fall within physiological limits. In view of the low predictive value of CER, it should be associated with the measurement of serum and urine copper for the diagnosis of Wilson’s disease.

مواضيع ذات صلة

Immunoglobulins

Complement

glucose tolerance test (GTT, Oral glucose tolerance test [OGTT])

glucose, postprandial (2-Hour postprandial glucose [2-Hour PPG], 1-Hour glucose screen)

α1-antitrypsin

Albumin

Diagnostic Laboratory Tests for Mycobacterium Tuberculosis

Glucose, blood (Blood sugar, Fasting blood sugar [FBS])

Gliadin, endomysial, and tissue transglutaminase antibodies

Antimicrobial Susceptibility Testing and Therapy of Staphylococcus, Micrococcus, and Similar Organisms

Glucagon

genetic testing (Breast cancer [BRCA] and ovarian cancer, Colon cancer, Cardiovascular disease, Tay–Sachs disease, Cystic fibrosis, Melanoma, Hemochromatosis, Thyroid cancer, Paternity [parentage analysis], Forensic genetic testing)