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Reduced Serotonin and 3-Hydroxyanthranilic Acid Levels in Serum of Cystatin B-Deficient Mice, a Model System for Progressive Myoclonus Epilepsy |
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| Authors: | Jelena Arbatova,&dagger &Dagger Elena D'Amato,§ Annika Vaarmann,§ Alex Zharkovsky, &# Mati Reeben |
| Affiliation: | Glenlab Niini Ltd., Helsinki, Finland;;Department of Pediatrics, II Clinic, University of Genoa;, and IRCCS Gaslini Children's Hospital, Genoa, Italy;;Department of Pharmacology, University of Tartu, Tartu, Estonia;, and Department of Basic Veterinary Medicine, University of Helsinki, Helsinki, Finland |
| Abstract: | Summary: Purpose: To evaluate the levels of tryptophan and its metabolites along serotonin (5-HT) and kynurenine (KYN) pathways in serum of progressive myoclonus epilepsy (EPM1) patients and cystatin B (CSTB)-deficient mice, a model system for EPM1. Methods: Tryptophan and its metabolites along serotonin (5-HT) and KYN pathways were determined in serum of EPM1 patients and CSTB-deficient mice by reverse-phase high-pressure liquid chromatography (HPLC) with electrochemical detection. Results: Reduced levels of 5-HT and KYN intermediate metabolite 3-hydroxyanthranilic acid were found in serum of CSTB-deficient mice. A similar trend was found in EPM1 patients. Although tryptophan concentration was reduced in serum of EPM1 patients, no such decrease was observed in CSTB-deficient mice. Conclusions: The present study demonstrates that tryptophan metabolism along 5-HT and KYN pathways are disrupted in EPM1. Further studies are needed to elucidate the role of KYN pathway in pathogenesis of EPM1. |
| Keywords: | Progressive myoclonus epilepsy Cystatin B-deficient mice Kynurenines Serotonin Tryptophan |