Fatty acid transport in multiple carboxylase deficiency fibroblasts.
Fatty acid transport in multiple carboxylase deficiency fibroblasts.
复制标题
多种羧化酶缺陷成纤维细胞中的脂肪酸转运。
DOI：
10.1007/bf01799574
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发表时间：
1990
影响因子：
4.2
通讯作者：
Whitney,S
中科院分区：
文献类型：
--
作者：
Packman,S;Whitney,S
全羧化酶合成酶（HS）和生物素酶缺乏症已被确定为生物素反应性多重羧化酶缺乏症的原因。乙酰辅酶A羧化酶（ACC）缺乏已被证明发生在多个羧化酶缺乏症，和HS（−）成纤维细胞被用来研究在ACC缺乏细胞的代偿性调节反应。在以前的研究中，生物素饥饿HS（−）成纤维细胞显示脂肪酸含量减少，脂肪酸的异常百分比组成，和细胞的长链脂肪酸含量的保存。我们在此询问突变体细胞是否显示出从培养基向成纤维细胞转运长链脂肪酸的代偿性增加。在本实验中，突变体和对照成纤维细胞在（+）或（-）生物素条件下生长后，花生四烯酸、棕榈酸或油酸的摄取没有变化。因此，从培养基中摄取不同的脂肪酸不是HS（-）细胞的补偿机制，也不能解释生物素限制产生的脂肪酸组成的特定变化。
Holocarboxylase synthetase (HS) and biotinidase deficiencies have been identified as causes of biotin-responsive multiple carboxylase deficiency. Acetyl-CoA carboxylase (ACC) deficiency has been shown to occur in multiple carboxylase deficiency, and HS(−) fibroblasts are being employed to investigate compensatory regulatory responses in cells deficient in ACC. In previous studies, biotin starved HS(−) fibroblasts showed a reduced fatty acid content, an abnormal percentage composition of fatty acids, and a preservation of longerchain fatty acid contents of cells. We herein ask whether the mutant cells show compensatory increases in the transport of longer-chain fatty acids from the medium into fibroblasts. In the present experiments there was no change in the uptake of arachidonate, palmitate or oleate following growth of mutant and control fibroblasts in (+) or (−) biotin conditions. Differential fatty acid uptake from the medium is therefore not a compensatory mechanism in HS(−) cells, and cannot account for the specific changes in fatty acid composition produced by biotin restriction.