| Literature DB >> 16997462 |
Flávia Karine Rigo1, Liana Pasquetti, Carlos Ricardo Maneck Malfatti, Michele Rechia Fighera, Rafael Correa Coelho, Cristiane Zanardo Petri, Carlos Fernando Mello.
Abstract
Propionic acid (PA) accumulates in patients with propionic acidemia, an inherited metabolic disorder caused by the deficiency of propionyl-CoA carboxylase activity that is clinically characterized by neurological dysfunction, including seizures. However, it is not known whether PA causes seizures in experimental animals. In the current study, we investigated whether intrastriatal injection of PA (0.6-6 micromol) causes seizures and alters protein carbonyl content in the striatum of adult rats. The injection of PA caused the appearance of seizures and increased protein carbonyl content in injected and noninjected striata. PA-induced seizures and increased protein carbonylation in the striatum were prevented by the injection of MK-801 (3 nmol/0.5 microL). Our results suggest that PA causes seizures and oxidative damage by NMDA receptor-mediated mechanisms. The involvement of NMDA receptors in the pathogenesis of propionic acidemia is suggested.Entities:
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Year: 2006 PMID： 16997462 DOI： 10.1016/j.neulet.2006.08.075
Source DB: PubMed Journal: Neurosci Lett ISSN： 0304-3940 Impact factor: 3.046