Brown W J, Warfel J, Greenspan P
Department of Pathology, University of South Carolina School of Medicine, Columbia 29208.
Arch Pathol Lab Med. 1988 Mar;112(3):295-7.
The fluorescent hydrophobic probe Nile red was used to distinguish between normal human fibroblasts and fibroblasts from individuals with a genetic deficiency in lysosomal acid lipase activity (Wolman's disease and cholesteryl ester storage disease). The fluorescence of Nile red-stained cultured mutant cells, indicative of neutral lipid accumulation, was intense when compared microscopically with normal fibroblasts. The cholesteryl ester accumulation in the acid lipase-deficient fibroblasts was demonstrated qualitatively and quantitatively when cellular lipid extracts were subjected to thin-layer chromatography, followed by Nile red plate treatment and fluorescence spectrometry scanning. These results demonstrate the utility of the Nile red stain to document cellular lipid overloading. The techniques are simple to perform and can effectively supplement the standard enzymatic analysis used in the diagnosis of acid lipase deficiency.
荧光疏水探针尼罗红被用于区分正常人成纤维细胞和溶酶体酸性脂肪酶活性存在基因缺陷个体（沃尔曼病和胆固醇酯贮积病）的成纤维细胞。与正常成纤维细胞相比，尼罗红染色的培养突变细胞的荧光（指示中性脂质积累）在显微镜下观察时很强。当细胞脂质提取物进行薄层色谱分析，随后进行尼罗红平板处理和荧光光谱扫描时，可定性和定量地证明酸性脂肪酶缺陷的成纤维细胞中胆固醇酯的积累。这些结果证明了尼罗红染色在记录细胞脂质过载方面的实用性。这些技术操作简单，可有效补充用于诊断酸性脂肪酶缺乏症的标准酶分析。
