Premium
Molecular Species of Phospholipids with Very Long Chain Fatty Acids in Skin Fibroblasts of Zellweger Syndrome
Author(s) -
Hama Kotaro,
Nagai Toru,
Nishizawa Chiho,
Ikeda Kazutaka,
Morita Masashi,
Satoh Noriko,
Nakanishi Hiroki,
Imanaka Tsuneo,
Shimozawa Nobuyuki,
Taguchi Ryo,
Inoue Keizo,
Yokoyama Kazuaki
Publication year - 2013
Publication title -
lipids
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.601
H-Index - 120
eISSN - 1558-9307
pISSN - 0024-4201
DOI - 10.1007/s11745-013-3848-5
Subject(s) - zellweger syndrome , phosphatidylethanolamine , phospholipid , peroxisome , phosphatidylcholine , biochemistry , adrenoleukodystrophy , fatty acid , plasmalogen , phosphatidylserine , biology , peroxisomal disorder , chemistry , gene , membrane
The ratio of C 26:0 /C 22:0 fatty acids in patient lipids is widely accepted as a critical clinical criterion of peroxisomal diseases, such as Zellweger syndrome and X‐linked adrenoleukodystrophy (X‐ALD). However, phospholipid molecular species with very long chain fatty acids (VLCFA) have not been precisely characterized. In the present study, the structures of such molecules in fibroblasts of Zellweger syndrome and X‐ALD were examined using LC–ESI–MS/MS analysis. In fibroblasts from Zellweger patients, a large number of VLCFA‐containing molecular species were detected in several phospholipid classes as well as neutral lipids, including triacylglycerol and cholesteryl esters. Among these lipids, phosphatidylcholine showed the most diversity in the structures of VLCFA‐containing molecular species. Some VLCFA possessed longer carbon chains and/or larger number of double bonds than C 26:0 ‐fatty acid (FA). Similar VLCFA were also found in other phospholipid classes, such as phosphatidylethanolamine and phosphatidylserine. In addition, VLCFA‐containing phospholipid species showed some differences among fibroblasts from Zellweger patients. It appears that phospholipids with VLCFA, with or without double bonds, as well as C 26:0 ‐FA might affect cellular functions, thus leading to the pathogenesis of peroxisomal diseases, such as Zellweger syndrome and X‐ALD.