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恶性贫血患者周围神经中异常的脂肪酸代谢。

Abnormal fatty acid metabolism in peripheral nerves of patients with pernicious anemia.

作者信息

Frenkel E P

出版信息

J Clin Invest. 1973 May;52(5):1237-45. doi: 10.1172/JCI107291.

Abstract

Fatty acid synthesis from radiopropionate was evaluated in sural nerve biopsy slices from five normal controls and nine patients with pernicious anemia. The nerves were incubated in [(14)C]propionate, the lipids were extracted, and the fatty acid methyl esters were chromatographed by gas-liquid chromatography. In the normal nerves the radiolabel was found primarily in short chain (C12 and C14) fatty acids. The nerves from pernicious anemia patients showed two fatty acids peaks that were not discernible in the normal nerves, and these fatty acids had retention times intermediate to those of myristic (C14.0) and palmitic (C16.0) acids and palmitoleic (C16.1) and stearic (C18.0) acids, respectively. These two peaks (a C15 and C17 fatty acid) contained the bulk of the radioactivity recovered in the fatty acid fraction after incubation with [(14)C]propionate. Catalytic reduction and rechromatography failed to alter the retention time of these compounds suggesting that they are not unsaturated fatty acids. The nerves from the pernicious anemia patients had a decrease in the mean content of normal fatty acids when compared with the nerves from control patients as well as a decrease in the mean synthesis of normal fatty acids as estimated by isotope incorporation after incubation with [(14)C]propionate or (3)H(2)O. Analysis of myelin isolated from the nerves indicated that the changes at least in part were in that fraction.

摘要

在取自5名正常对照者和9名恶性贫血患者的腓肠神经活检切片中评估了由放射性丙酸合成脂肪酸的情况。将神经在[¹⁴C]丙酸中孵育,提取脂质,并用气-液色谱法对脂肪酸甲酯进行色谱分析。在正常神经中,放射性标记主要存在于短链(C12和C14)脂肪酸中。恶性贫血患者的神经显示出两个在正常神经中无法辨别的脂肪酸峰,这些脂肪酸的保留时间分别介于肉豆蔻酸(C14.0)和棕榈酸(C16.0)以及棕榈油酸(C16.1)和硬脂酸(C18.0)之间。这两个峰(一种C15脂肪酸和一种C17脂肪酸)包含了与[¹⁴C]丙酸孵育后在脂肪酸部分中回收的大部分放射性。催化还原和重新色谱分析未能改变这些化合物的保留时间,这表明它们不是不饱和脂肪酸。与对照患者的神经相比,恶性贫血患者的神经中正常脂肪酸的平均含量降低,并且在用[¹⁴C]丙酸或³H₂O孵育后通过同位素掺入估计的正常脂肪酸平均合成量也降低。对从神经中分离出的髓磷脂的分析表明,至少部分变化发生在该部分。

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