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Elevated plasma nociceptin level in patients with Wilson disease
Mónika B Hantos1, Ferenc Szalay, Péter L Lakatos
1Department of Pharmacodynamics, Semmelweis University, Budapest, Hungary.
Brain Research Bulletin
|July 20, 2002
Summary
Plasma nociceptin levels are significantly higher in Wilson disease patients due to copper toxicity impacting enzymes that inactivate nociceptin.
Area of Science:
- Biochemistry
- Genetics
- Neurology
Background:
- Wilson disease is an inherited disorder of copper metabolism caused by ATP7B gene mutations.
- Toxic copper accumulation in organs like the liver and brain characterizes Wilson disease.
- Nociceptin is an endogenous peptide and agonist of the orphanin FQ/ORL1 receptor.
Purpose of the Study:
- To investigate plasma nociceptin levels in patients with Wilson disease.
- To explore the relationship between plasma nociceptin and disease indicators.
Main Methods:
- Plasma nociceptin levels were measured using 125I-radioimmunoassay.
- Wilson disease patients (n=20) were compared to age-matched healthy controls (n=25).
- Correlation with liver function tests and analysis of sex differences were performed.
Main Results:
- Plasma nociceptin levels were significantly elevated in Wilson disease patients (13.98±2.44 pg/ml) versus controls (9.18±1.63 pg/ml; p<0.001).
- No significant correlation was found between plasma nociceptin levels and liver function tests.
- No sex-based differences in plasma nociceptin levels were observed.
Conclusions:
- Elevated plasma nociceptin in Wilson disease may result from copper-induced inhibition of nociceptin-inactivating enzymes.
- Inhibition of aminopeptidase N (APN) and endopeptidase 24.15 by toxic copper deposits is a potential mechanism.
- This finding suggests a novel biochemical pathway affected in Wilson disease.