翻訳と辞書 Words near each other ・ Long-tailed fantail ・ Long-tailed finch ・ Long-tailed fiscal ・ Long-tailed forest shrew ・ Long-tailed fruit bat ・ Long-billed spiderhunter ・ Long-billed starthroat ・ Long-billed thrasher ・ Long-billed thrush ・ Long-billed white-eye ・ Long-billed woodcreeper ・ Long-billed wren ・ Long-billed wren (New Zealand) ・ Long-billed wren-babbler ・ Long-bolt engine ・ Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency ・ Long-chain acyl-(acyl-carrier-protein) reductase ・ Long-chain acyl-CoA dehydrogenase ・ Long-chain-alcohol dehydrogenase ・ Long-chain-alcohol O-fatty-acyltransferase ・ Long-chain-alcohol oxidase ・ Long-chain-aldehyde dehydrogenase ・ Long-chain-enoyl-CoA hydratase ・ Long-chain-fatty-acid—(acyl-carrier-protein) ligase ・ Long-chain-fatty-acid—CoA ligase ・ Long-chain-fatty-acid—luciferin-component ligase ・ Long-chain-fatty-acyl-CoA reductase ・ Long-chain-fatty-acyl-glutamate deacylase ・ Long-clawed ground squirrel ・ Long-clawed mole vole Dictionary Lists mini英和辞書 mini和英辞書 Webster 1913 Latin-English FOLDOC Wikipedia English ウィキペディア

翻訳と辞書　辞書検索 [ 開発暫定版 ] スポンサード リンク Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency ： ウィキペディア英語版 Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency, often shortened to LCHAD deficiency, is a rare autosomal recessive fatty acid oxidation disorder that prevents the body from converting certain fats into energy. This can become life-threatening, particularly during periods of fasting. ==Genetics== Mutations in the ''HADHA'' gene lead to inadequate levels of an enzyme called long-chain 3-hydroxyacyl-coenzyme A (CoA) dehydrogenase, which is part of a protein complex known as mitochondrial trifunctional protein. Long-chain fatty acids from food and body fat cannot be metabolized and processed without sufficient levels of this enzyme. As a result, these fatty acids are not converted to energy, which can lead to characteristic features of this disorder, such as lethargy and hypoglycemia. Long-chain fatty acids or partially metabolized fatty acids may build up in tissues and damage the liver, heart, retina, and muscles, causing more serious complications. 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency」の詳細全文を読む スポンサード リンク 翻訳と辞書 : 翻訳のためのインターネットリソース Copyright(C) kotoba.ne.jp 1997-2016. All Rights Reserved.