{"title":"Analytical Methods for Quantitative Plasma Carnitine Determination.","authors":"Chelsea N Zimmerman, J Daniel Sharer","doi":"10.1002/cphg.85","DOIUrl":null,"url":null,"abstract":"<p><p>Carnitine is an essential molecule for mitochondrial beta-oxidation of long-chain fatty acids and other cellular functions. Several rare, inherited disorders of carnitine metabolism occur in humans, and secondary carnitine deficiency is an important feature in a variety of clinical settings. Many of these conditions can be detected via quantitative analysis of free and esterified carnitine in plasma or urine, which thus offers an effective means for assessing the transport and initial processing of fatty acids. Here, we describe some of the methods most commonly employed for quantification of plasma carnitine and consider some of the advantages and disadvantages of these approaches. © 2019 by John Wiley & Sons, Inc.</p>","PeriodicalId":40007,"journal":{"name":"Current Protocols in Human Genetics","volume":"102 1","pages":"e85"},"PeriodicalIF":0.0000,"publicationDate":"2019-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1002/cphg.85","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Current Protocols in Human Genetics","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1002/cphg.85","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
Abstract
Carnitine is an essential molecule for mitochondrial beta-oxidation of long-chain fatty acids and other cellular functions. Several rare, inherited disorders of carnitine metabolism occur in humans, and secondary carnitine deficiency is an important feature in a variety of clinical settings. Many of these conditions can be detected via quantitative analysis of free and esterified carnitine in plasma or urine, which thus offers an effective means for assessing the transport and initial processing of fatty acids. Here, we describe some of the methods most commonly employed for quantification of plasma carnitine and consider some of the advantages and disadvantages of these approaches. © 2019 by John Wiley & Sons, Inc.
血浆肉碱定量测定的分析方法。
肉碱是线粒体β -氧化长链脂肪酸和其他细胞功能所必需的分子。一些罕见的,遗传性的肉毒碱代谢疾病发生在人类中,继发性肉毒碱缺乏是各种临床环境中的一个重要特征。许多这些条件可以通过定量分析血浆或尿液中的游离和酯化肉碱来检测,从而为评估脂肪酸的运输和初始加工提供了有效的手段。在这里,我们描述了一些最常用于定量血浆肉碱的方法,并考虑了这些方法的一些优点和缺点。©2019 by John Wiley & Sons, Inc。
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