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ceruloplasmin

Biochemistry/HepatologyHepaticNervous SystemOphthalmologicHematologic

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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ceruloplasmin — Medical Glossary