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Methylmalonic and propionic acidemias: lipid profiles of normal and affected human skin fibroblasts incubated with [1-14C]propionate.

作者信息

Giudici T A, Chen R G, Oizumi J, Shaw K N, Ng W G, Donnell G N

出版信息

Biochem Med Metab Biol. 1986 Jun;35(3):384-98. doi: 10.1016/0885-4505(86)90097-6.

DOI:10.1016/0885-4505(86)90097-6
PMID:2872907
Abstract

Normal human skin fibroblasts and those from methylmalonic acidemia and propionic acidemia patients were grown in culture. Following incubation with [1-14C]propionate, the major lipid classes in the cells were separated by thin layer chromatography and isolated fractions analyzed by radio gas chromatography for the presence of odd-numbered long-chain fatty acids; the pattern of even-numbered long-chain fatty acids was obtained also. Normal fibroblasts incorporated a small percentage of propionate into odd-numbered fatty acids which were present in all lipids studied. The abnormal cells incorporated a larger amount while maintaining the characteristic ratios of odd-numbered fatty acids found in the normal line. Most of the radioactivity was associated with phospholipids which are the predominant constituents of cell membranes. A characteristic C15/C17 ratio was found for different phospholipids and the triglyceride fraction; pentadecanoic acid was the principal odd-numbered fatty acid utilized in the assembly of complex lipids. Compared to even-numbered long-chain fatty acids the absolute amount of odd-numbered fatty acids was low (1-2%), even in affected cells. An unusual polar lipid fraction was isolated in the course of the study. In the normal cell it contained several unlabeled eicosanoids which were missing from the same fraction of both affected cell lines.

摘要

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2
Odd-numbered long-chain fatty acid contents in erythrocyte membrane phospholipids in patients with an impaired propionate utilization.
J Inherit Metab Dis. 1988;11 Suppl 2:225-8. doi: 10.1007/BF01804242.
3
Primary structure and activity of mouse methylmalonyl-CoA mutase.小鼠甲基丙二酰辅酶A变位酶的一级结构与活性
Biochem J. 1990 Oct 15;271(2):449-55. doi: 10.1042/bj2710449.