Essential role of ELOVL4 protein in very long chain fatty acid synthesis and retinal function

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题名:	Essential role of ELOVL4 protein in very long chain fatty acid synthesis and retinal function
作者:	Harkewicz, R.a, Du, H.bcd, Tong, Z.b, Alkuraya, H.b, Bedell, M.b, Sun, W.b, Wang, X.be, Hsu, Y.-H.a, Esteve-Rudd, J.f, Hughes, G.b, Su, Z.c, Zhang, M.c, Lopes, V.S.f, Molday, R.S.g, Williams, D.S.f, Dennis, E.A.a , Zhang, K.b
贡献者:	Department of Chemistry, Tunghai University
日期:	2012
上传时间:	2013-06-11T09:05:33Z (UTC)
摘要:	Very long chain polyunsaturated fatty acid (VLC-PUFA)-containing glycerophospholipids are highly enriched in the retina; however, details regarding the specific synthesis and function of these highly unusual retinal glycerophospholipids are lacking. Elongation of very long chain fatty acids-4 (ELOVL4) has been identified as a fatty acid elongase protein involved in the synthesis of VLC-PUFAs. Mutations in ELOVL4 have also been implicated in an autosomal dominant form of Stargardt disease (STGD3), a type of juvenile macular degeneration. We have generated photoreceptor-specific conditional knock-out mice and used high performance liquid chromatography-mass spectrometry (HPLC-MS) to examine and analyze the fatty acid composition of retinal membrane glycerophosphatidylcholine and glycerophosphatidylethanolamine species. We also used immunofluorescent staining and histology coupled with electrophysiological data to assess retinal morphology and visual response. The conditional knock-out mice showed a significant decrease in retinal glycerophospholipids containing VLC-PUFAs, specifically contained in the sn-1 position of glycerophosphatidylcholine, implicating the role of Elovl4 in their synthesis. Conditional knock-out mice were also found to have abnormal accumulation of lipid droplets and lipofuscin-like granules while demonstrating photoreceptor-specific abnormalities in visual response, indicating the critical role of Elovl4 for proper rod or cone photoreceptor function. Altogether, this study demonstrates the essential role of ELOVL4 in VLC-PUFA synthesis and retinal function. ? 2012 by The American Society for Biochemistry and Molecular Biology, Inc.
關聯:	Journal of Biological Chemistry Volume 287, Issue 14, 30 March 2012, Pages 11469-11480
显示于类别:	[化學系所] 期刊論文

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