Functions of plasmalogen lipids in health and disease

被引:794
作者
Braverman, Nancy E. [1 ]
Moser, Ann B. [2 ]
机构
[1] McGill Univ, Montreal Childrens Hosp, Res Inst, Dept Human Genet & Pediat, Montreal, PQ H3Z 2Z3, Canada
[2] Johns Hopkins Univ Hosp, Kennedy Krieger Inst, Dept Neurogenet, Baltimore, MD 21287 USA
来源
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE | 2012年 / 1822卷 / 09期
关键词
Plasmalogen; Rhizomelic Chondrodysplasia Punctata; Alzheimer disease; Respiratory disease; Lipid signaling; Plasmalogen replacement therapy; RHIZOMELIC CHONDRODYSPLASIA PUNCTATA; ALKYL-DIHYDROXYACETONEPHOSPHATE-SYNTHASE; TANDEM MASS-SPECTROMETRY; ALZHEIMERS-DISEASE; FATTY-ACID; PEROXISOMAL DISORDERS; ZELLWEGER-SYNDROME; OXIDATIVE STRESS; ARACHIDONIC-ACID; CHIMYL ALCOHOL;
D O I
10.1016/j.bbadis.2012.05.008
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Plasmalogens are a unique class of membrane glycerophospholipids containing a fatty alcohol with a vinyl-ether bond at the sn-1 position, and enriched in polyunsaturated fatty acids at the sn-2 position of the glycerol backbone. These two features provide novel properties to these compounds. Although plasmalogens represent up to 20% of the total phospholipid mass in humans their physiological roles have been challenging to identify, and are likely to be particular to different tissues, metabolic processes and developmental stages. Their biosynthesis starts in peroxisomes, and defects at these steps cause the malformation syndrome, Rhizomelic Chondrodysplasia Punctata (RCDP). The RCDP phenotype predicts developmental roles for plasmalogens in bone, brain, lens, lung, kidney and heart. Recent studies have revealed secondary plasmalogen deficiencies associated with more common disorders and allow us to tease out additional pathways dependent on plasmalogen functions. In this review, we present current knowledge of plasmalogen biology in health and disease. This article is part of a Special Issue entitled: Metabolic Functions and Biogenesis of peroxisomes in Health and Disease. (C) 2012 Elsevier B.V. All rights reserved.
引用
收藏
页码:1442 / 1452
页数:11
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