红细胞膜中极长链脂肪酸和缩醛磷脂的分析：一种检测过氧化物酶体疾病以及区分肾上腺脑白质营养不良和泽韦格综合征的简便方法。

Analysis of very long-chain fatty acids and plasmalogen in the erythrocyte membrane: a simple method for the detection of peroxisomal disorders and discrimination between adrenoleukodystrophy and Zellweger syndrome.

作者信息

Tanaka K, Nishizawa K, Yamamoto H, Naruto T, Izeki E, Taga T, Shimada M, Saeki Y

机构信息

Department of Pediatrics, Nagahama Red Cross Hospital, Japan.

出版信息

Neuropediatrics. 1990 Aug;21(3):119-23. doi: 10.1055/s-2008-1071476.

DOI:10.1055/s-2008-1071476

PMID:2234315

Abstract

We analyzed the sphingomyelin very long-chain fatty acids (VLCFAs) and phosphatidylethanolamine (PE) plasmalogen contents of the erythrocyte membrane in patients suffering from peroxisomal disorders. In a patient with Zellweger syndrome, both a decrease in the PE plasmalogen content and an increase in the sphingomyelin VLCFAs content of the erythrocyte membrane were noted. In patients with adrenoleukodystrophy, however, there was no decrease in PE plasmalogen, although the sphingomyelin VLCFAs content of the membrane was significantly increased in comparison with control values. Analyses of both sphingomyelin VLCFAs and PE plasmalogen were carried out simultaneously, using both the same process and the same sample.

摘要

我们分析了患有过氧化物酶体疾病患者红细胞膜中的鞘磷脂超长链脂肪酸（VLCFAs）和磷脂酰乙醇胺（PE）缩醛磷脂含量。在一名患有泽尔韦格综合征的患者中，发现红细胞膜中PE缩醛磷脂含量降低，鞘磷脂VLCFAs含量增加。然而，在肾上腺脑白质营养不良患者中，尽管与对照值相比膜中鞘磷脂VLCFAs含量显著增加，但PE缩醛磷脂含量并未降低。使用相同的方法和相同的样本同时对鞘磷脂VLCFAs和PE缩醛磷脂进行了分析。