کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8260240 | 1534656 | 2014 | 10 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Mitochondrial bioenergetics deregulation caused by long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies in rat brain: A possible role of mPTP opening as a pathomechanism in these disorders?
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کلمات کلیدی
N-[2-hydroxyethyl]piperazine-N′-[2-ethane-sulfonic acid]fluorescence arbitrary unitscarbonyl cyanide 3-chlorophenyl hydrazineFAUHEPESEGTALCHADALMLCFAantimycin AMTPMPTPCCCPAlamethicin - AlamethicBSA - BSAROS - ROSbovine serum albumin - آلبومین سرم گاوethylene glycol-bis(2-aminoethylether)-N,N,N′,N′-tetraacetic acid - اتیلن گلیکول بیست (2-aminoethylether) -N، N، N '، N'-tetraacetic acidLong-chain fatty acids - اسیدهای چرب بلند زنجیره ایPermeability transition pore - افت فشار نفوذ پذیریmitochondrial permeability transition pore - انتقال نفوذی میتوکندری منفی استCSA - ایالات مؤتلفهٔ آمریکاruthenium red - رتنیم قرمزblood brain barrier - سد خونی مغزیBBB - سد خونی مغزیcyclosporin A - سیکلوسپورین AMitochondrial trifunctional protein - پروتئین trifunctional میتوکندریCalcium - کلسیمReactive oxygen species - گونههای فعال اکسیژن
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
سالمندی
پیش نمایش صفحه اول مقاله
چکیده انگلیسی
Long-chain 3-hydroxylated fatty acids (LCHFA) accumulate in long-chain 3-hydroxy-acyl-CoA dehydrogenase (LCHAD) and mitochondrial trifunctional protein (MTP) deficiencies. Affected patients usually present severe neonatal symptoms involving cardiac and hepatic functions, although long-term neurological abnormalities are also commonly observed. Since the underlying mechanisms of brain damage are practically unknown and have not been properly investigated, we studied the effects of LCHFA on important parameters of mitochondrial homeostasis in isolated mitochondria from cerebral cortex of developing rats. 3-Hydroxytetradecanoic acid (3 HTA) reduced mitochondrial membrane potential, NAD(P)H levels, Ca2Â + retention capacity and ATP content, besides inducing swelling, cytochrome c release and H2O2 production in Ca2Â +-loaded mitochondrial preparations. We also found that cyclosporine A plus ADP, as well as ruthenium red, a Ca2Â + uptake blocker, prevented these effects, suggesting the involvement of the mitochondrial permeability transition pore (mPTP) and an important role for Ca2Â +, respectively. 3-Hydroxydodecanoic and 3-hydroxypalmitic acids, that also accumulate in LCHAD and MTP deficiencies, similarly induced mitochondrial swelling and decreased ATP content, but to a variable degree pending on the size of their carbon chain. It is proposed that mPTP opening induced by LCHFA disrupts brain bioenergetics and may contribute at least partly to explain the neurologic dysfunction observed in patients affected by LCHAD and MTP deficiencies.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease - Volume 1842, Issue 9, September 2014, Pages 1658-1667
Journal: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease - Volume 1842, Issue 9, September 2014, Pages 1658-1667
نویسندگان
Anelise Miotti Tonin, Alexandre Umpierrez Amaral, Estela Natacha Busanello, Juciano Gasparotto, Daniel P. Gelain, Niels Gregersen, Moacir Wajner,