Long-term experience with triheptanoin in 12 Austrian patients with long-chain fatty acid oxidation disorders

机译：在12名奥地利长链脂肪酸氧化疾病中患有三肽蛋白的长期经验

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Diagram of the mitochondrial metabolic LC-FAOD and C7 pathways. Abbreviations: CPT1: carnitine palmitoyltransferase 1; CPT2: carnitine palmitoyltransferase 2; VLCAD: very long-chain acyl-CoA dehydrogenase; MCAD: medium-chain acyl-CoA dehydrogenase; SCAD: short-chain acyl-CoA dehydrogenase; LCHAD: long-chain 3-hydroxyacyl-CoA dehydrogenase; SCHAD: short-chain 3-hydroxylacyl-CoA dehydrogenase; LCKAT: long-chain 3-ketothiolase; MCKAT: medium-chain 3-ketoacyl-CoA thiolase; PROP-CoA: propionyl-CoA; FAO: fatty acid oxidation; CAC: citric acid cycle; ATP: adenosine triphosphate. Copyright Thomas Zöggeler MD

机译：线粒体代谢LC-FAOD和C7途径的图。缩写：CPT1：肉毒氨基丙酰棕榈酰转移酶1; CPT2：肉毒碱棕榈酰棕榈酰转移酶2; VLCAD：非常长链酰基 - COA脱氢酶; MCAD：中链酰基 - COA脱氢酶;斯科德：短链酰基-CoA脱氢酶; Lchad：长链3-羟基乙酰-CoA脱氢酶; Schad：短链3-羟基 - COA脱氢酶; Lckat：长链3-酮酶; McKat：中链3-酮酰基-COA硫酶; PROP-COA：丙酰基COA;粮农组织：脂肪酸氧化; CAC：柠檬酸循环; ATP：腺苷三磷酸。版权所有ThomasZöggelerMD

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期刊名称 Orphanet Journal of Rare Diseases
作者
Thomas Zöggeler; Katharina Stock; Monika Jörg-Streller; Johannes Spenger; Vassiliki Konstantopoulou; Miriam Hufgard-Leitner; Sabine Scholl-Bürgi; Daniela Karall;
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年(卷),期 2021(-1),-1
年度 2021
页码 -1
总页数 9
原文格式 PDF
正文语种
中图分类医学史;
关键词
Inherited metabolic disorders; Inborn errors of metabolism; Anaplerotic effect; Fat intake regimen; Long-chain ß-oxidation disorders;

机译：遗传性代谢障碍;天生的新陈代谢误差;厌氧作用;脂肪摄入方案;长链β-氧化障碍;

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1. Odd or even? Results from a randomized trial of triheptanoin compared to MCT in patients with long-chain fatty acid oxidation disorders [J] . Gillingham Melanie B., Harding Cary O., Vockley Jerry Molecular genetics and metabolism . 2015,第3期

机译：奇数或偶数？长链脂肪酸氧化紊乱患者中三庚酸与MCT的随机试验结果
2. Long-term major clinical outcomes in patients with long chain fatty acid oxidation disorders before and after transition to triheptanoin treatment-A retrospective chart review (vol 116, pg 53, 2015) [J] . Vockley Jerry, Marsden Deborah, McCracken Elizabeth, Molecular genetics and metabolism . 2015,第3期

机译：长链脂肪酸氧化紊乱患者在过渡到三庚酸治疗前后的长期主要临床结局-回顾性图表回顾（第116卷，第53页，2015年）
3. Long-term major clinical outcomes in patients with long chain fatty acid oxidation disorders before and after transition to triheptanoin treatment-A retrospective chart review [J] . Vockley Jerry, Marsden Deborah, McCracken Elizabeth, Molecular genetics and metabolism . 2015,第1a2期

机译：过渡到三庚酸治疗前后长链脂肪酸氧化紊乱患者的长期主要临床结局-回顾性图表回顾
4. INFRARED STUDY ON ORDER-DISORDER TRANSITION IN LANGMUIR-BLODGETT FILMS OF AZOBEZENE-CONTAINING LONG-CHAIN FATTY ACIDS [C] . Yuqing Wu, Bing Zhao, Weiqing Xu, The 2nd Asian symposium on organized molecular films for electronics and photonics : Abstract book . 1998

机译：含偶氮苯长链脂肪酸的朗缪尔-布洛杰德膜中有序-有序转变的红外研究
5. Long-chain polyunsaturated fatty acid intake and its relationship to red blood cell and plasma long-chain polyunsaturated fatty acids in women at high risk for breast cancer. [D] . Harvey, Katherine E. 2010

机译：罹患乳腺癌高风险女性的长链多不饱和脂肪酸摄入及其与红细胞和血浆长链多不饱和脂肪酸的关系。
6. Triheptanoin versus trioctanoin for long-chain fatty acid oxidation disorders: a double blinded randomized controlled trial [O] . Melanie B. Gillingham, Stephen B. Heitner, Julie Martin, -1

机译：三庚酸与三辛酸治疗长链脂肪酸氧化疾病：一项双盲随机对照试验
7. Long-term Experience with Triheptanoin in 12 Austrian Patients with Long-chain Fatty Acid Oxidation Disorders [O] . Thomas Zöggeler, Katharina Stock, Monika Jörg-Streller, 2020

机译：在12名奥地利长链脂肪酸氧化疾病中患有三肽蛋白的长期经验
8. Synthesis and Biological Evaluation of (E)-19-Iodo-3,3-Dimethyl-18-Nonadecenoic Acid, a New Dimethyl-Branched Long-Chain Fatty Acid to Evaluate Regional Myocardial Fatty Acid Uptake [R] . Goodman, M. M., Ambrose, K. R., Neff, K. H., 1986

机译：（E）-19-碘-3,3-二甲基-18-十一碳烯酸（一种新的二甲基支链长链脂肪酸）的合成和生物学评价，用于评估局部心肌脂肪酸的摄取

Long-term experience with triheptanoin in 12 Austrian patients with long-chain fatty acid oxidation disorders

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