کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
9880338 | 1535226 | 2005 | 11 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Intrahippocampal administration of the branched-chain α-hydroxy acids accumulating in maple syrup urine disease compromises rat performance in aversive and non-aversive behavioral tasks
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کلمات کلیدی
BCKABranched chain keto acidsMSUDHMVbranched chain amino acids - آمینو اسیدهای زنجیرهای منشعب شدهinhibitory avoidance - اجتناب مهار کنندهHIC - اینBCAA - بی سی ای ایMaple syrup urine disease - بیماری ادرار شربت افراCNS - دستگاه عصبی مرکزیRat behavior - رفتار موشcentral nervous system - سیستم عصبی مرکزیOpen field - میدان بازHIV - ویروس نقص ایمنی انسانی GABA - گابا
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
سالمندی
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چکیده انگلیسی
Maple syrup urine disease (MSUD) is an inherited metabolic disease predominantly characterized by neurological dysfunction. Although a variable degree of psychomotor/delay/mental retardation is found in a considerable number of MSUD patients, the mechanisms underlying the neuropathology of this disorder are yet not defined. The present study investigated the effect of acute intrahippocampal administration of the branched-chain α-hydroxy acids (BCHA) accumulating in MSUD on rat behavior in non-aversive (open field) and aversive (inhibitory avoidance) tasks. Cannulated 60-day-old male Wistar rats received bilateral intrahippocampal injection of α-hydroxyisocaproic acid (HIC, 1.5 μmol), α-hydroxyisovaleric acid (HIV, 2.5 μmol), α-hydroxy-β-methyl-n-valeric acid (HMV, 1.5 μmol), or NaCl (2.5 μmol)(controls) immediately after or 10 min before training. Testing session was performed 24 h later. Administration of the hydroxy acids immediately after training caused no effect on the cognitive performance of the rats. In contrast, HIV and HMV administered 10 min before training provoked a habituation deficit in the open field task. Motor activity, assessed by crossing responses, was the same for the groups infused with BCHA and NaCl. The effect of MK-801, succinate, creatine, and the antioxidants ascorbic acid plus α-tocopherol on the behavioral alterations provoked by HIV in the open field task revealed that only the energetic substrates (succinate and creatine) prevented these effects, reflecting a possible compromise of brain energy production by HIV. We also observed that rats pretreated with HIC, HIV, or HMV did not increase their latency in the testing session in the step-down inhibitory avoidance task, revealing an impairment of retrieval (memory retention or acquisition) in this task. Furthermore, no differences between controls and rats receiving BCHA were detected in the latency to leave the platform in the training test, suggesting similar motor activity of all groups. The data indicate that the α-hydroxy acids accumulating in MSUD impair cognition and may be implicated in the neuropathology and psychomotor delay/mental retardation observed in the affected patients.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Journal of the Neurological Sciences - Volume 232, Issues 1â2, 15 May 2005, Pages 11-21
Journal: Journal of the Neurological Sciences - Volume 232, Issues 1â2, 15 May 2005, Pages 11-21
نویسندگان
Vilson de Castro Vasques, FabrÃcio Brinco, Moacir Wajner,