Erdorf, Miriam and von der Ohe, Juliane and Seifert, Roland (2011) Impaired P2X and P2Y receptor-mediated signaling in HPRT-deficient B103 neuroblastoma cells. NEUROSCIENCE LETTERS, 504 (3). pp. 311-315. ISSN 0304-3940, 1872-7972
Full text not available from this repository. (Request a copy)Abstract
Defect of hypoxanthine phosphoribosyl transferase (HPRT) causes Lesch-Nyhan disease (LND), but the link between HPRT deficiency and the self-injurious behavior of LND is unknown. In a previous study (Pinto et al., J. Neurochem. 72 (2005) 1579-1586) we reported on a decrease in nucleotidase activity in membranes of several HPRT- cell lines and fibroblasts from LND patients. Since nucleotidases are involved in ATP-induced signal transduction, in the present study, we tested the hypothesis that P2X and P2Y receptor-mediated signal transduction is impaired in HPRT deficiency. As model we studied rat B103 neuroblastoma cells. Compared to control cells, in HPRT- cells, NTP and NDP-induced Ca2+ influx across the membrane and Ca2+ mobilization from intracellular stores were impaired. Both P2X and P2Y receptors were involved in the responses. Quantitative real-time PCR revealed reduced expression of receptors P2X(3), P2X(5), P2Y(2), P2Y(4), P2Y(12), P2Y(13) and P2Y(14) in HPRT deficiency. Collectively, HPRT deficiency is associated with abnormal purinergic signaling, encompassing P2X and P2Y receptors and nucleotidases. (C) 2011 Elsevier Ireland Ltd. All rights reserved.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | LESCH-NYHAN-DISEASE; PURINE; MODEL; PHARMACOLOGY; FIBROBLASTS; METABOLISM; DISORDERS; MICE; B103 neuroblastoma cells; Hypoxanthine phosphoribosyl transferase; LND; Lesch-Nyhan disease; Purinoceptors; Adenine nucleotides |
| Subjects: | 600 Technology > 615 Pharmacy |
| Divisions: | Chemistry and Pharmacy > Institute of Pharmacy > Pharmacology and Toxicology (Prof. Schlossmann, formerly Prof. Seifert) |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 28 May 2020 14:00 |
| Last Modified: | 28 May 2020 14:00 |
| URI: | https://pred.uni-regensburg.de/id/eprint/19950 |
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