Tsuji S, Ohno T, Miyatake T, Suzuki A, Yamakawa T
J Biochem. 1984 Oct;96(4):1241-7. doi: 10.1093/oxfordjournals.jbchem.a134942.
The activities of microsomal fatty acid elongation and cytoplasmic de novo fatty acid synthesis were measured in human cultured skin fibroblasts. Both activities in fibroblasts from normal controls and patients with adrenoleukodystrophy (ALD) were compared and slight but a significant increase of elongation activities in ALD fibroblasts was demonstrated. On the other hand, there were no significant differences in the fatty acid synthetase activities. In this study, we measured microsomal fatty acid elongation activities in the presence of N-ethylmaleimide, which completely inhibited the activity of contaminating fatty acid synthetase and also the degradation of fatty acids, and made accurate determination of the elongation activities possible.
在人培养的皮肤成纤维细胞中测量微粒体脂肪酸延长和细胞质从头脂肪酸合成的活性。比较了正常对照和成肾上腺脑白质营养不良（ALD）患者成纤维细胞中的这两种活性，结果表明ALD成纤维细胞中的延长活性有轻微但显著的增加。另一方面，脂肪酸合成酶活性没有显著差异。在本研究中，我们在N-乙基马来酰亚胺存在的情况下测量微粒体脂肪酸延长活性，N-乙基马来酰亚胺完全抑制了污染的脂肪酸合成酶的活性以及脂肪酸的降解，从而使得准确测定延长活性成为可能。
