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正常及过氧化物酶体缺陷(泽尔韦格综合征)成纤维细胞中由[2-¹⁴C]乙酸盐合成脂肪酸的过程。

Fatty acid synthesis from [2-14C]acetate in normal and peroxisome-deficient (Zellweger) fibroblasts.

作者信息

Poulos A, Usher S, Paton B C

机构信息

Department of Chemical Pathology, Women's and Children's Hospital, North Adelaide, South Australia.

出版信息

Lipids. 1993 Feb;28(2):97-102. doi: 10.1007/BF02535771.

DOI:10.1007/BF02535771
PMID:8441343
Abstract

The incorporation of [2-14C]acetate into the lipids of normal and peroxisome-deficient (Zellweger's syndrome) skin fibroblasts was examined. Most of the label was incorporated into triacylglycerol fatty acids in normal as well as Zellweger's syndrome cells. Triacylglycerols and cholesteryl esters in Zellweger's syndrome cells contained increased levels of labelled saturated and monounsaturated very long-chain fatty acids (VLCFA, that is fatty acids with more than 22 carbon atoms), in particular hexacosanoic (26:0) and hexacosaenoic (26:1) acids. As traces of labelled VLCFA with up to 32 carbon atoms were detected in triacylglycerols even in control cells it is probable that these fatty acids are formed naturally during the elongation process. Our data suggest that peroxisomes are involved in the chain shortening of the saturated and monounsaturated VLCFA.

摘要

研究了[2-¹⁴C]乙酸盐掺入正常和过氧化物酶体缺陷(泽尔韦格综合征)皮肤成纤维细胞脂质中的情况。在正常细胞以及泽尔韦格综合征细胞中,大部分标记物掺入了三酰甘油脂肪酸中。泽尔韦格综合征细胞中的三酰甘油和胆固醇酯含有标记的饱和和单不饱和极长链脂肪酸(VLCFA,即碳原子数超过22的脂肪酸)水平升高,特别是二十六烷酸(26:0)和二十六碳烯酸(26:1)。即使在对照细胞的三酰甘油中也检测到了含32个碳原子的标记VLCFA痕迹,因此这些脂肪酸很可能是在延长过程中自然形成的。我们的数据表明,过氧化物酶体参与了饱和和单不饱和VLCFA的链缩短过程。

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