ceruloplasmin
Summary
Ceruloplasmin is a copper-carrying glycoprotein synthesized by the liver that transports 90-95% of plasma copper. Decreased levels are pathognomonic for Wilson disease, while elevated levels occur in inflammatory conditions and pregnancy.
Detail
Ceruloplasmin is an acute-phase reactant and ferroxidase enzyme that plays crucial roles in copper transport and iron metabolism. It carries 6-7 copper atoms per molecule and facilitates iron oxidation from Fe2+ to Fe3+, enabling iron binding to transferrin. In Wilson disease (hepatolenticular degeneration), a defect in the ATP7B gene impairs copper incorporation into ceruloplasmin, resulting in low serum ceruloplasmin levels (<20 mg/dL) and toxic copper accumulation in the liver, brain, and cornea (Kayser-Fleischer rings). Ceruloplasmin levels increase during inflammation, infection, pregnancy, and estrogen therapy due to its acute-phase response properties. The protein has a half-life of 5-6 days and is essential for preventing oxidative damage through its antioxidant properties. Laboratory measurement is critical for Wilson disease diagnosis, though 5-15% of Wilson disease patients may have normal ceruloplasmin levels.
Sources
- Robbins and Cotran Pathologic Basis of Disease
- Harrison's Principles of Internal Medicine
- First Aid for the USMLE Step 1
- Biochemistry (Lippincott's Illustrated Reviews)
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