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神经酰胺的高效液相色谱法:在人体组织分析中的应用及法伯病中神经酰胺过量的证明

High performance liquid chromatography of ceramides: application to analysis in human tissues and demonstration of ceramide excess in Farber's disease.

作者信息

Sugita M, Iwamori M, Evans J, McCluer R H, Dulaney J T, Moser H W

出版信息

J Lipid Res. 1974 May;15(3):223-6.

PMID:4363967
Abstract

Conditions have been determined for the benzoylation of ceramides containing nonhydroxy and hydroxy fatty acids, and a high performance liquid chromatography system for the separation and measurement of these derivatives has been devised that is capable of good resolution and high sensitivity. These methods have been used to determine quantitatively the levels of ceramides in human tissues, and in serum and urine, and to demonstrate elevated amounts of ceramide in Farber's disease urine and tissues.

摘要

已确定含有非羟基和羟基脂肪酸的神经酰胺进行苯甲酰化的条件,并设计了一种用于分离和测量这些衍生物的高效液相色谱系统,该系统具有良好的分辨率和高灵敏度。这些方法已用于定量测定人体组织、血清和尿液中的神经酰胺水平,并证明法伯病患者尿液和组织中的神经酰胺含量升高。

相似文献

1
High performance liquid chromatography of ceramides: application to analysis in human tissues and demonstration of ceramide excess in Farber's disease.神经酰胺的高效液相色谱法:在人体组织分析中的应用及法伯病中神经酰胺过量的证明
J Lipid Res. 1974 May;15(3):223-6.
2
Fatty acid composition of free ceramides of kidney and cerebellum from a patient with Farber's disease.法伯病患者肾脏和小脑游离神经酰胺的脂肪酸组成。
Lipids. 1973 Jul;8(7):401-6. doi: 10.1007/BF02531715.
3
Ceramidase and ceramide synthesis in human kidney and cerebellum. Description of a new alkaline ceramidase.人肾和小脑中的神经酰胺酶与神经酰胺合成。一种新型碱性神经酰胺酶的描述。
Biochim Biophys Acta. 1975 Jul 22;398(1):125-31. doi: 10.1016/0005-2760(75)90176-9.
4
Farber's disease as a ceramidosis: clinical, radiological and biochemical aspects.法伯病作为一种神经酰胺沉积症:临床、放射学及生物化学方面
Acta Paediatr Scand. 1978 Jan;67(1):113-9. doi: 10.1111/j.1651-2227.1978.tb16287.x.
5
Above-normal urinary excretion of urinary ceramides in Farber's disease, and characterization of their components by high-performance liquid chromatography.法布里病患者尿神经酰胺排泄高于正常水平及其成分的高效液相色谱表征
Clin Chem. 1975 May;21(6):725-9.
6
Ceramides in a patient with lipogranulomatosis (Farber's disease) with chronic course.一名患有慢性病程的脂肪肉芽肿病(法伯病)患者体内的神经酰胺
Scand J Clin Lab Invest. 1971 Jun;27(4):393-405. doi: 10.3109/00365517109080235.
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[Farber's disease (disseminated lipogranulomatosis) (author's transl)].法伯氏病(播散性脂肪肉芽肿病)(作者译)
Monatsschr Kinderheilkd (1902). 1974 Apr;122(4):153-9.
8
Analysis and quantitation of free ceramide containing nonhydroxy and 2-hydroxy fatty acids, and phytosphingosine by high-performance liquid chromatography.通过高效液相色谱法分析和定量含有非羟基和2-羟基脂肪酸的游离神经酰胺以及植物鞘氨醇。
J Lipid Res. 1979 Jan;20(1):86-96.
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[Farber's disease. Anatomoclinical and ultrastructural study].[法伯氏病。解剖临床及超微结构研究]
Bull Acad R Med Belg. 1974 Oct;13(9):733-62.
10
The cellular lesions of Farber's disease and their experimental reproduction in tissue culture.法伯病的细胞病变及其在组织培养中的实验性再现。
Lab Invest. 1977 May;36(5):474-80.

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Biomolecules. 2023 Feb 1;13(2):274. doi: 10.3390/biom13020274.
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探测和评估哺乳动物神经酰胺代谢的方法。
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Acid ceramidase deficiency: Farber disease and SMA-PME.酸性神经酰胺酶缺乏症:法伯病和 SMA-PME。
Orphanet J Rare Dis. 2018 Jul 20;13(1):121. doi: 10.1186/s13023-018-0845-z.
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C26-Ceramide as highly sensitive biomarker for the diagnosis of Farber Disease.C26-神经酰胺可作为法伯病诊断的高度敏感生物标志物。
Sci Rep. 2017 Jul 21;7(1):6149. doi: 10.1038/s41598-017-06604-2.
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Acid Ceramidase Deficiency in Mice Results in a Broad Range of Central Nervous System Abnormalities.小鼠酸性神经酰胺酶缺乏导致广泛的中枢神经系统异常。
Am J Pathol. 2017 Apr;187(4):864-883. doi: 10.1016/j.ajpath.2016.12.005.
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A simple fluorogenic method for determination of acid ceramidase activity and diagnosis of Farber disease.一种用于测定酸性神经酰胺酶活性和法伯病诊断的简单荧光法。
J Lipid Res. 2010 Dec;51(12):3542-7. doi: 10.1194/jlr.D010033. Epub 2010 Sep 24.
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Synthesis, NMR characterization and divergent biological actions of 2'-hydroxy-ceramide/dihydroceramide stereoisomers in MCF7 cells.2'-羟基神经酰胺/二氢神经酰胺立体异构体在 MCF7 细胞中的合成、NMR 表征及不同的生物学作用。
Bioorg Med Chem. 2010 Nov 1;18(21):7565-79. doi: 10.1016/j.bmc.2010.08.050. Epub 2010 Sep 28.
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Fatty acid 2-Hydroxylation in mammalian sphingolipid biology.哺乳动物鞘脂生物学中的脂肪酸2-羟基化作用
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Myocardium of type 2 diabetic and obese patients is characterized by alterations in sphingolipid metabolic enzymes but not by accumulation of ceramide.2 型糖尿病和肥胖患者的心肌表现为鞘脂代谢酶的改变,但没有神经酰胺的积累。
J Lipid Res. 2010 Jan;51(1):74-80. doi: 10.1194/jlr.M900002-JLR200.