Abstract

1. It has been suggested that low levels of serum caeruloplasmin in Wilson's disease result from the failure to switch from a fetal to an adult mode of caeruloplasmin gene expression. To investigate postnatal expression of the caeruloplasmin gene, steady-state levels of caeruloplasmin messenger RNA in adult and neonatal rat liver were measured. 2. Copper parameters observed in neonatal rats were similar to those seen in Wilson's disease: hepatic copper concentration was significantly elevated (neonatal 164 +/- 35 micrograms/g, adults 50 +/- 8 micrograms/g, P less than .001) and serum copper and caeruloplasmin levels were low (neonatal 0.5 +/- 0.1 microgram/ml, adults 1.3 +/- 0.2 microgram/ml, P less than .001; neonatal 0.20 +/- 0.04 arbitrary units, adults 0.69 +/- 0.16 arbitrary units, P less than .001), respectively. 3. Caeruloplasmin messenger RNA levels were analysed by Northern and dot blotting using a 32P-labelled caeruloplasmin complementary DNA probe. A caeruloplasmin messenger RNA of approximately 4.4 kilobases was detected in both adult and neonatal rat liver, with no significant difference observed in steady-state levels. 4. A step subsequent to caeruloplasmin gene transcription must therefore be impaired in neonatal rats.

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