Disturbance of mitochondrial functions provoked by the major long-chain 3-hydroxylated fatty acids accumulating in MTP and LCHAD deficiencies in skeletal muscle
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- 作者：Cristiane Cecattoa ; Ká ; lita dos Santos Godoya ; Janaí ; na Camacho da Silvaa ; Alexandre Umpierrez Amarala ; Moacir Wajnera ; b ; mwajner@ufrgs.br" class="auth_mail" title="E-mail the corresponding author
- 关键词：ANT ; adenine nucleotide translocator ; ATC ; atractyloside ; BSA ; bovine serum albumin ; CCCP ; carbonyl cyanide 3-chlorophenyl hydrazine ; CsA ; cyclosporin A ; EGTA ; ethylene glycol-bis (2-aminoethylether)-N ; N ; N&prime ; N&prime ; -tetraacetic acid ; EtOH ; ethanol ; FAU ; fluorescence arbitrary units ; GDP ; guanosine diphosphate ; HEPES ; N-[2-hydroxyethyl]piperazine-N&prime ; -[2-ethane-sulfonic acid] ; 3HTDA ; 3-hydroxytetradecanodioic acid ; 3HTA ; 3-hydroxytetradecanoic acid ; 3HPA ; 3-hydroxypalmitic acid ; LCFA ; l
- 刊名：Toxicology in Vitro
- 出版年：2016
- 出版时间：October 2016
- 年：2016
- 卷：36
- 期：Complete
- 页码：1-9
- 全文大小：892 K
文摘
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3-Hydroxy long-chain fatty acids (LCHFA) accumulate in LCHAD and MTP deficiencies.
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LCHFA disrupt energy and Ca+ homeostasis in muscle mitochondria of young rats.
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LCHFA compromise respiration in skeletal muscle mitochondria and permeabilized fibers.
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20">3HPA effects in muscle mitochondria cause mPT induction.
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25">These pathomechanisms may be involved in the muscle symptoms of these patients.