Abstract
Extract: Radioimmunoassay can detect as little as 10 ng apoceruloplasmin. Interference by holoceruloplasmin is overcome by using an antiapoceruloplasmin antibody absorbed with holoceruloplasmin and by correcting for the relatively small inhibition of binding of apoceruloplasmin to absorbed antibody caused by holoceruloplasmin. The apoceruloplasmin concentration in infants with nutritional copper deficiency (2.9 ± 0.9 mg/100 ml), Menkes' kinky hair syndrome (1.4 ± 0.5 mg/100 ml), and Wilson's disease (2.7 ± 2.0 mg/100 ml) did not differ significantly from normal (3.3 ± 3.1 mg/100 ml) despite significant reductions (P < 0.01) in the concentration of holoceruloplasmin in each of these conditions. In umbilical cord blood from normal neonates, however, the concentration of apoceruloplasmin (0.7 ± 0.5 mg/100 ml) was significantly lower than normal (P < 0.01), as was the concentration of holoceruloplasmin.
Speculation: In Wilson's disease the incorporation of copper into nascent apoceruloplasmin, which is probably synthesized at a normal rate and is of normal structure, is reduced. There could be a defect in an enzyme required to catalyze the incorporation or a failure of copper to reach the nascent protein.
In normal neonates the rate of apoceruloplasmin synthesis is probably reduced. This reduction may be responsible for the lower concentrations of both holo- and apoceruloplasmin observed in sera of neonates compared with that of older subjects.
Similar content being viewed by others
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Matsuda, I., Pearson, T. & Holtzman, N. Determination of Apoceruloplasmin by Radioimmunoassay in Nutritional Copper Deficiency, Menkes' Kinky Hair Syndrome, Wilson's Disease, and Umbilical Cord Blood. Pediatr Res 8, 821–824 (1974). https://doi.org/10.1203/00006450-197410000-00001
Issue Date:
DOI: https://doi.org/10.1203/00006450-197410000-00001
Keywords
This article is cited by
-
Hepatic and Hippocampus Iron Status is not Altered in Response to Increased Serum Ceruloplasmin and Serum “Free” Copper in Wistar Rat Model for Non-Wilsonian Brain Copper Toxicosis
Biological Trace Element Research (2013)
-
Truncating mutations in the Wilson disease gene ATP7B are associated with very low serum ceruloplasmin oxidase activity and an early onset of Wilson disease
BMC Gastroenterology (2010)
-
Measurement of blood holoceruloplasmin by EIA using a mouse monoclonal antibody directed to holoceruloplasmin. Implication for mass screening of Wilson disease
Journal of Inherited Metabolic Disease (1994)
-
Menkes' kinky hair disease
Neuroradiology (1977)