肾上腺脑白质营养不良（ALD）和肾上腺脊髓神经病（AMN）患者培养的皮肤成纤维细胞中[17,18 - 3H2]二十六烷酸和[15,16 - 3H2]木蜡酸的代谢

Metabolism of [17,18-3H2]hexacosanoic acid and [15,16-3H2]lignoceric acid in cultured skin fibroblasts from patients with adrenoleukodystrophy (ALD) and adrenomyeloneuropathy (AMN).

作者信息

Tsuji S, Sano-Kawamura T, Ariga T, Miyatake T

出版信息

J Neurol Sci. 1985 Dec;71(2-3):359-67. doi: 10.1016/0022-510x(85)90074-7.

Abstract

We have studied the metabolism of [17,18-3H2]hexacosanoic acid and [15,16-3H2]lignoceric acid in cultured skin fibroblasts from patients with adrenoleukodystrophy (ALD) and adrenomyeloneuropathy (AMN). The successful solubilization of the very long chain fatty acids as albumin conjugates was confirmed by analysis using glycerol density gradient centrifugation. The composition of radioactive fatty acids in total lipids of fibroblasts after culture for 7 days in the presence of [17,18-3H2] hexacosanoic acid or [15,16-3H2]lignoceric acid was analyzed by radio-gas chromatography. Most of the fatty acids added to the culture media were degraded to shorter-chain fatty acids both by control, and ALD or AMN fibroblasts. However, the content of the remaining radioactive hexacosanoic acid or lignoceric acid was much higher in ALD or AMN fibroblasts, and the content of radioactive shorter-chain fatty acids was much lower in ALD or AMN fibroblasts. These results indicate that the degradation of very long chain fatty acids is decreased in ALD, but also that there is a substantial amount of residual activity of their degradation in ALD fibroblasts.

摘要

我们研究了肾上腺脑白质营养不良（ALD）和肾上腺脊髓神经病（AMN）患者培养的皮肤成纤维细胞中[17,18 - 3H₂]十六烷酸和[15,16 - 3H₂]二十四烷酸的代谢。通过甘油密度梯度离心分析证实了超长链脂肪酸作为白蛋白缀合物的成功增溶。在[17,18 - 3H₂]十六烷酸或[15,16 - 3H₂]二十四烷酸存在下培养7天后，通过放射性气相色谱分析成纤维细胞总脂质中放射性脂肪酸的组成。添加到培养基中的大多数脂肪酸在对照、ALD或AMN成纤维细胞中均降解为短链脂肪酸。然而，ALD或AMN成纤维细胞中剩余放射性十六烷酸或二十四烷酸的含量要高得多，而ALD或AMN成纤维细胞中放射性短链脂肪酸的含量要低得多。这些结果表明，ALD中超长链脂肪酸的降解减少，但ALD成纤维细胞中其降解仍存在大量残余活性。