کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
10832997 | 1065780 | 2013 | 10 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
In vivo experimental evidence that the major metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency induce oxidative stress in striatum of developing rats: A potential pathophysiological mechanism of striatal damage in this disorder
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کلمات کلیدی
GPXN-methyl-d-aspartateRNSDCFH-DADCFHSPSSGSSGMELMGAHmgHMGANACGSH3-methylglutaric acidNMDATBA-RS3-hydroxy-3-methylglutaric aciduriaDcfCATG6PDDNPHTCA2′,7′-dichlorofluorescein - 2 '، 7'-dichlorofluorescein2′,7′-dichlorofluorescin diacetate - 2 '، 7'-dichlorofluorescin diacetate2,4-dinitrophenylhydrazine - 2،4-dinitrophenylhydrazineGC/MS - GC / MSl-NAME - L-NAMENω-nitro-l-arginine methyl ester - N-Nitro-L-Arginine Methyl EsterN-acetylcysteine - N-استیل سیستئینROS - ROSStriatum - استریاتوم3-hydroxy-3-methylglutaric acid - اسید 3-هیدروکسی-3-متیل گلوتاریکtrichloroacetic acid - اسید ترشکلراکتیکStatistical Package for the Social Sciences - بسته های آماری برای علوم اجتماعیOxidative stress - تنش اکسیداتیوSOD - سدSuperoxide dismutase - سوکسوکس دیسموتازMelatonin - ملاتونینCatalase - کاتالازreduced glutathione - کاهش گلوتاتیونgas chromatography/mass spectrometry - کروماتوگرافی گاز / طیف سنج جرمیoxidized glutathione - گلوتاتیون اکسید شدهglutathione reductase - گلوتاتیون ردوکتازglutathione peroxidase - گلوتاتیون پراکسیدازglucose-6-phosphate dehydrogenase - گلوکز 6-فسفات دهیدروژنازreactive nitrogen species - گونه های واکنش پذیر نیتروژنReactive oxygen species - گونههای فعال اکسیژن
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
زیست شیمی
پیش نمایش صفحه اول مقاله
چکیده انگلیسی
3-Hydroxy-3-methylglutaryl-CoA lyase (HL) deficiency is a genetic disorder biochemically characterized by predominant accumulation of 3-hydroxy-3-methylglutaric (HMG) and 3-methylglutaric (MGA) acids in tissues and biological fluids of affected individuals. Clinically, the patients present neurological symptoms and basal ganglia injury, whose pathomechanisms are partially understood. In the present study, we investigated the ex vivo effects of intrastriatal administration of HMG and MGA on important parameters of oxidative stress in striatum of developing rats. Our results demonstrate that HMG and MGA induce lipid and protein oxidative damage. HMG and MGA also increased 2â²,7â²-dichlorofluorescein oxidation, whereas only HMG elicited nitric oxide production, indicating a role for reactive oxygen (HMG and MGA) and nitrogen (HMG) species in these effects. Regarding the enzymatic antioxidant defenses, both organic acids decreased reduced glutathione concentrations and the activities of superoxide dismutase and glutathione reductase and increased glutathione peroxidase activity. HMG also provoked an increase of catalase activity and a diminution of glucose-6-phosphate dehydrogenase activity. We finally observed that antioxidants fully prevented or attenuated HMG-induced alterations of the oxidative stress parameters, further indicating the participation of reactive species in these effects. We also observed that MK-801, a non-competitive antagonist of the N-methyl-D-aspartate (NMDA) receptor, prevented some of these effects, indicating the involvement of the NMDA receptor in HMG effects. The present data provide solid evidence that oxidative stress is induced in vivo by HMG and MGA in rat striatum and it is presumed that this pathomechanism may explain, at least in part, the cerebral alterations observed in HL deficiency.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Molecular Genetics and Metabolism - Volume 109, Issue 2, June 2013, Pages 144-153
Journal: Molecular Genetics and Metabolism - Volume 109, Issue 2, June 2013, Pages 144-153
نویسندگان
Carolina Gonçalves Fernandes, Mateus Struecker da Rosa, Bianca Seminotti, Paula Pierozan, Rafael Wolter Martell, Valeska Lizzi Lagranha, Estela Natacha Brandt Busanello, Guilhian Leipnitz, Moacir Wajner,