کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
4339808 | 1295768 | 2009 | 10 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Evidence for catabolic pathway of propionate metabolism in CNS: expression pattern of methylmalonyl-CoA mutase and propionyl-CoA carboxylase alpha-subunit in developing and adult rat brain
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کلمات کلیدی
MUTPropionic aciduriaMethylmalonyl-CoA mutasePCCApropionyl-CoA carboxylaseMCMmethylmalonic acidISHPCCNeuNDEPCGFAPPFAMMAMBPTCAHRPIn situ hybridization - Hybridization در محلbranched chain amino acids - آمینو اسیدهای زنجیرهای منشعب شدهtricarboxylic acid - اسید تری کربوکسیلیکBCAA - بی سی ای ایDeveloping brain - توسعه مغزdiethylpyrocarbonate - دیاتیلپیر کربناتMethylmalonic aciduria - متیل مالون اسیدوریاparaformaldehyde - پارافرمالدهیدhorse radish peroxidase - پراکسیداز تربچه اسبGlial fibrillary acidic protein - پروتئین اسیدی فیبریلاسیون گلایالneuronal nuclei protein - پروتئین هسته ای نورونMyelin basic protein - پروتئین پایه میلین
موضوعات مرتبط
علوم زیستی و بیوفناوری
علم عصب شناسی
علوم اعصاب (عمومی)
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چکیده انگلیسی
Methylmalonyl-CoA mutase (MCM) and propionyl-CoA carboxylase (PCC) are the key enzymes of the catabolic pathway of propionate metabolism and are mainly expressed in liver, kidney and heart. Deficiency of these enzymes leads to two classical organic acidurias: methylmalonic and propionic aciduria. Patients with these diseases suffer from a whole spectrum of neurological manifestations that are limiting their quality of life. Current treatment does not seem to effectively prevent neurological deterioration and pathophysiological mechanisms are poorly understood. In this article we show evidence for the expression of the catabolic pathway of propionate metabolism in the developing and adult rat CNS. Both, MCM and PCC enzymes are co-expressed in neurons and found in all regions of the CNS. Disease-specific metabolites such as methylmalonate, propionyl-CoA and 2-methylcitrate could thus be formed autonomously in the CNS and contribute to the pathophysiological mechanisms of neurotoxicity. In rat embryos (E15.5 and E18.5), MCM and PCC show a much higher expression level in the entire CNS than in the liver, suggesting a different, but important function of this pathway during brain development.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Neuroscience - Volume 164, Issue 2, 1 December 2009, Pages 578-587
Journal: Neuroscience - Volume 164, Issue 2, 1 December 2009, Pages 578-587
نویسندگان
D. Ballhausen, L. Mittaz, O. Boulat, L. Bonafé, O. Braissant,