cis-4-Decenoic and decanoic acids impair mitochondrial energy, redox and Ca2 + homeostasis and induce mitochondrial permeability transition pore opening in rat brain and liver: Possible implications for the pathogenesis of MCAD deficiency
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- 作者：Alexandre Umpierrez Amarala ; Cristiane Cecattoa ; Janaí ; na Camacho da Silvaa ; Alessandro Wajnera ; Ká ; lita dos Santos Godoya ; Rafael Teixeira Ribeiroa ; Moacir Wajnera ; b ; mwajner@ufrgs.br" class="auth_mail" title="E-mail the corresponding author
- 关键词：AA ; antimycin A ; ANT ; adenine nucleotide translocator ; ATC ; atractyloside ; BSA ; bovine serum albumin ; CCCP ; carbonyl cyanide 3-chlorophenyl hydrazine ; cDA ; cis-4-decenoic acid ; CsA ; cyclosporin A ; DA ; decanoic acid ; DC ; decanoylcarnitine ; EGTA ; ethylene glycol-bis (2-aminoethylether)-N ; N ; N&prime ; N&prime ; -tetraacetic acid ; EtOH ; ethanol ; FAU ; fluorescence arbitrary units ; GP ; generalized polarization ; HEPES ; N-[2-hydroxyethyl]piperazine-N&prime ; -[2-ethane-sulfonic acid] ; MDA ; malondialdehyde ; MC
- 刊名：Biochimica et Biophysica Acta (BBA) - Bioenergetics
- 出版年：2016
- 出版时间：September 2016
- 年：2016
- 卷：1857
- 期：9
- 页码：1363-1372
- 全文大小：1142 K
文摘
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MCFA and carnitine derivatives accumulate in MCAD deficiency.
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DA and cDA are metabolic inhibitors, uncouplers and mPT inducers in brain and liver.
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DA and cDA disturb calcium and redox homeostasis in brain and liver.
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20">These mechanisms may be involved in the pathophysiology of this disorder.