Like any other plasma protein, levels drop in patients with hepatic disease due to reduced synthesizing capabilities.
Mechanisms of low ceruloplasmin levels:
* Gene expression genetically low (aceruloplasminemia)
* Copper levels are low in general
** Malnutrition/trace metal deficiency in the food source
** Zinc toxicity, due to induced copper deficiency
* Copper does not cross the intestinal barrier due to ATP7A deficiency (Menkes disease and Occipital horn syndrome)
* Delivery of copper into the lumen of the ER-Golgi network is absent in hepatocytes due to absent ATP7B (Wilson's disease)
Copper availability doesn't affect the translation of the nascent protein. However, the apoenzyme without copper is unstable. Apoceruloplasmin is largely degraded intracellularly in the hepatocyte and the small amount that is released has a short circulation half life of 5 hours as compared to the 5.5 days for the holo-ceruloplasmin.
Ceruloplasmin can be measured by means of a blood test; this can be done using immunoassays . The sample is spun and separated; it is stored around 4°C Celsius for three days. This test is to determine if there are signs of Wilson disease. Another test that can be done is a urine copper level test; this has been found to be less accurate than the blood test. A liver tissue test can be done as well.
Mutations in the ceruloplasmin gene (CP), which are very rare, can lead to the genetic disease aceruloplasminemia, characterized by hyperferritinemia with iron overload. In the brain, this iron overload may lead to characteristic neurologic signs and symptoms, such as cerebellar ataxia, progressive dementia, and extrapyramidal signs. Excess iron may also deposit in the liver, pancreas, and retina, leading to cirrhosis, endocrine abnormalities, and loss of vision, respectively.